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CONGENITAL  CARDIAC   DISEASE 


B^ 


MAUDE  E.  ABBOTT,  B.A.,  M.D. 

MCGILL  UNIVEESITT,  MONTREAL,    CANADA 


BEPRINTED  FROM 

OSLER  &  McCRAE'S  MODERN  MEDICINE 
Vol.  IV,  2d  Edition,  1915 


Lea  &  Febiger 
Philadelphia  and  New  York 


'R  CC8:3 


CHAPTER  X. 
CONGENITAL  CARDIAC  DISEASE.^ 

By  MAUDE  E.  ABBOTT,  M.D. 

Definition. — Congenital  cardiac  disease  may  be  defined  as  that  condition 
in  which,  through  arrest  of  development  or  disease  occurring  in  intra- 
uterine life,  anomalies  in  the  anatomical  structure  of  the  heart  or  great 
vessels  exist,  leading  to  irregularities  in  the  circulation.  It  is  frequently 
associated  with  congenital  cyanosis  and  clubbing  of  the  fingers,  and  con- 
stitutes in  extreme  cases  the  morbus  ccBndeus  of  the  older  -^Titers. 

THE  DEVELOPMENT  OF  THE  HEART. 

It  is  impossible  to  approach  this  subject  intelligently  without  a  certain 
preliminary  knowledge  of  the  development  of  the  mammalian  heart.  A 
brief  statement  referring  especially  to  the  development  of  the  septa,  the 
involution  of  the  bulbus  cordis  and  sinus  venosus,  and  the  disappearance  of 
the  primitive  aortic  arches,  is  therefore  necessary  here.  For  fuller  details 
the  reader  is  referred  to  the  fundamental  studies  of  His^  and  Born^  and  to 
the  recent  contributions  of  Tandler,^  Monckeberg,^  and  Mall.*^ 

The  mammalian  heart  is  formed  originally  of  two  straight  tubes  placed 
independently  on  either  side  of  the  body,  which  merge  together  as  the 
ventral  cleft  closes  in  and  finally  fuse,  the  septum  thus  formed  becoming 
entirely  obliterated  before  the  permanent  interventricular  septum  begins 
to  appear.  Meanwhile  a  twisting  of  the  heart  upon  its  long  axes  occurs, 
and  it  becomes  no  longer  symmetrical,  but  S-shaped,  with  the  ventricular 
portion  bent  forward  and  downward  and  the  auricular  part  upward  and 
backward.  It  now  consists  of  two  chambers,  a  single  ventricle  forming 
its  anterior  and  lower  part  with  its  bulbus  cordis  passing  upward  and 
to  the  left  and  giving  off  the  aortic  trunk  from  its  right  angle  (Figs.  16  and 
17),  and  a  single  auricle  with  its  sinus  venosus  lying  behind  and  to  the 
left.  At  this  stage  it  resembles  the  two-chambered  heart  of  the  fish,  and 
is  especially  interesting  in  regard  to  the  formation  of  the  bulbus  cordis. 

1  This  article  has  been  largely  rewritten,  and  curtailed  in  parts,  to  permit  of  the 
addition  of  new  material,  especially  under  the  Development  of  the  Heart,  Anomalies 
of  the  Pericardium,  Dextrocardia,  Congenital  Rhabdomyoma,  Auricular,  Ventricular, 
and  Aortic  Septal  Defects,  Deviation  of  the  Aortic  Septum,  and  Patent  Ductus 
Arteriosus.  The  reader  is  referred  to  the  earlier  edition  for  the  omitted  material. 
-  2  Beitrdge  zur  Anatomie  des  menschlichen  Herzens,  Leipzig,  1886. 
-^   ^  Beitrage  zur  Entwickelung  des  Saugethierherzens,  Arch,  f .  miki*.  anat.,  1889,  xxxiii. 

^  Keibel  and  Mall's  Human  Embryology,  1912,  ii,  pp.  534-570. 

^  Atlas  der  Herzmissbildungen,  Jena,  1912;  also,  Verh.  d.  Deut.  Path.  GeselL,  Marburg, 
1913,  xvi,  p.  228. 

6  Am.  Jour.  Anat.,  1913,  xiv,  p.  249. 

(323) 


324 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


The  auricle  next  shifts  upward,  coming  to  He  above  the  ventricle,  and  its 
auricular  appendages  develop  enormously,  pouching  forward  on  either  side 
of  the  bulbus  (Fig.  19).     The  atrial  canal,  in  which  are  developing  the 


Fig.  16 


Fig.  17 


Fig.  18 


The  separation  of  the  ventricles.     (After  His.) 

Fig.  16. — Embryo  1.9  mm.  long.  The  single  ventricle  gives  off  the  common  arterial  trunk  (B)  from 
its  right  upper  angle,  and  receives  the  common  auriculoventricular  orifice  (F.au.c.)  entirely  on  its  left  side. 

Fig.  17. — Embryo  3.5  mm.  long.  The  common  trunk  has  moved  somewhat  to  the  left  and  is  divided 
by  the  aortic  septum.  The  interventricular  septum  (S.iv.)  divides  the  ventricle  into  two  parts,  leaving 
the  interventricular  foramen  (O.iv.)  still  open  above.  The  auriculoventricular  orifice  (F.au.c.)  has 
moved  to  the  right,  so  that  part  of  it  lies  in  each  cavity.  E.o.,  upper  endocardial  cushion;  E.u.,  lower 
endocardial  cushion;  T'.rf.,  right  ventricle;  V.s  left  ventricle. 

Fig.  18. — The  aortic  and  interventricular  septa  have  united,  and  completed  the  division  of  the  ventricles. 
The  pulmonarj-  arterj^  (Ar.p.)  arises  from  the  right,  the  aorta  (Ao.)  from  the  left  ventricle,  and  the  right 
and  left  auriculoventricular  ostia  (F.au.d.,  F.au.s.)  lie  in  their  respective  cavities.  V.d.,  right  ventricle; 
V.S.,  left  ventricle;  S.iv.,  interventricular  septum.     (From  Vierordt,  Nothnagel's  Series,  xv,  1-2.) 


endocardial  cushions  which  are  to  separate  the  two  venous  ostia,  has 
become  elongated  and  still  opens  into  the  common  ventricle  entirely  on  the 
left  side.  The  sinus  venosus  is  now  a  separate  cavity  opening  into  the 
auricle  on  its  right  wall  posteriorly  through  a  narrow  cleft,  the  edges  of 


PLATE  V 


Model  of  the  Heart  of  a  Human  Embryo  4.6  mm.  long  x  108. 
F.  T.   Lewis  and  M.  E.  Abbott.     (Dr.   Begg's  Embryo.) 

{From  the  Anatomical  Laboratory  of  the  Harvard  Medical  School.) 

C,  carotid  arch;  P. A  ,  pulmonary  artery;  Per.,  pericardium;  Tr.A.,  truncus  arteriosus; 
A.d.,  right  auricle;  A.s.,  left  auricle;  S.v.,  sinus  venosus;  Au.c,  common  auriculoventricular 
jrifice;  B.v,,  bulboventricular  cleft;  V.,  common  ventricle. 


CONGENITAL  CARDIAC  DISEASE  325 

which  project  into  the  auricle  as  the  valvulse  venosse  dextra  et  sinistra. 
At  its  upper  border  it  is  elongated  laterally  into  the  two  sinus  horns 
which  receive  the  two  superior  venae  cavse,  while  a  single  short  trunk,  the 
inferior  cava,  enters  it  below. 

The  Bulbus  Cordis. — This  name  is  given  to  a  transitory  portion  of  the 
embryonic  heart  leading  from  the  right  end  of  the  common  ventricle  to 
the  aortic  arches.  In  the  human  embryo  of  4  to  6  mm.  in  length  the 
bulbus  is  a  thick- walled  muscular  tube  passing  to  the  left  and  upward,  lined 
like  the  rest  of  the  heart  with  endothelium,  which  presents  certain  endo- 
cardial thickenings,  spirally  arranged  (Tandler),  the  so-called  proximal  and 
distal  bulbar  swellings,  structures  which  later  form  the  a^ilagen  of  the 
semilunar  cusps  as  well  as  of  the  lower  part  of  the  aortopulmonary  sep- 
tum. In  later  stages  the  bulbus  disappears,  its  proximal  portion  being 
taken  up  in  the  wall  of  the  ventricle,  and  its  distal  part,  denuded  of  its 
musculature  and  considerably  elongated,  constituting  the  primitive  aortic 
trunk.  The  researches  of  GreiP  on  the  reptilian  heart,  and  Keith,-  and 
recently  of  Jane  Robertson^  on  the  fish,  show  that  the  mammalian  bulbus 
represents  w^hat  was  at  one  time  an  independent  chamber  with  muscular 
walls  and  its  own  system  of  multiple  valves,  w^hich  in  the  "ontogenetic 
telescoping  of  phylogenetic  stages"  has  become  submerged. 

Robertson  correlates  her  findings  in  the  fish  with  those  of  Greil  in  the 
lizard,  and  Born  in  the  mammalian  embryo,  and  traces  the  bulbus  of  the 
latter  back  through  the  less  blurred  stages  of  the  reptile  to  the  simpler 
forms  seen  in  the  Dipnoan  and  Elasmobranch  fishes.  Thus  this  structure, 
represented  in  the  adult  mammal,  with  its  fully  established  double  circu- 
lation, by  the  completely  separated  aortic  and  pulmonary  trunks,  is  seen 
in  Lacerta  (reptile)  to  consist  of  a  curved  muscular  tube  divided  by  a  spiral 
aortopulmonary  septum,  wdiich  gives  way  in  turn  in  Lepidosiren  (Dipnoan 
fish)  to  a  kinked  muscular  tube  with  median  expansion  incompletely 
divided  by  rows  of  spirally  arranged  valves,  and  this  again  in  the  Elasmo- 
branch fishes  with  their  purely  branchial  respiration,  is  reduced  to  the 
simplest  form  as  a  straight  channel  with  muscular  walls  lined  by  numerous 
rows  of  longitudinally  placed  valves.  These  phylogenetic  proofs  of  an 
early  bulbar  channel  with  spiral  division  of  its  distal  portion,  are  of  the 
utmost  importance  in  the  elucidation  of  the  problems  of  stenosis  of  the 
pulmonary  conus  and  transposition  of  the  arterial  trunks,  and  yield 
striking  confirmation  of  the  explanations  offered  by  Keith  of  the  former, 
and  by  Rokitansky  of  the  latter  anomaly. 

The  Interauricidar  Septum. — Born  showed  that  the  division  of  the 
auricles  takes  place  through  the  development  of  two  different  partitions 
placed  in  planes  parallel  with  each  other  developing  successively,  parts  of 
both  of  which  are  temporary,  while  parts  persist  to  form  the  permanent 
interauricular  septum  of  postnatal  life.  Of  these  septa,  the  one  developing 
earlier,  called  by  Born  the  septum  primiim,  begins  about  the  fourth  week 
from  the  upper  and  posterior  wall  of  the  auricle  as  a  sickle-shaped  fold 
which  grows  forward  and  downward  toward  the  ventricular  cavity,  and 

1  Morph.  Jahrb.,  1903,  xxxi,  p.  123. 

-  Festschrift  of  the  Quatercentenary  of  Aberdeen  University,  July,  1906. 

'  Jortr.  Path,  and  Bacterial.,  1913,  xviii,  p.  191. 


326 


DISEASES  OF  THE  CIRCULATORY  SYSTEM 


for  some  time  an  opening  exists  between  the  auricles  at  the  lower  border  of 
this  primitive  septum  known  as  the  ostium  primum.  About  the  begin- 
ning of  the  fifth  week  a  second  opening,  called  by  Born  the  ostium 
secundiun,  forms  in  the  now  greatly  thinned  upper  and  back  part  of  the 
septum  primum.  This  second  opening  grows  larger  as  the  ostium  primum 
becomes  smaller,  and  finally  disappears  entirely  (end  of  fifth  week), 
through  the  union  of  the  expanded  lower  margin  of  the  septum  primum 
with  the  fused  endocardial  cushions  between  the  auriculoventricular 
ostia.  There  thus  exists  a  stage  in  development  when  the  septum 
primum  is  represented  by  a  band  of  tissue  between  two  orifices,  the  ostium 
secundum  above,  and  the  ostium  primum  below.     (See  Figs.  19  and  20.) 


Fig.  19 


A.d. 


'J>  V'.  _ 


Transverse  section  through  the  heart  region  of  an  embryo  of  8  mm.  greatest  length.  A.d.,  descending 
aorta;  Au.,  atrial  canal;  S.,  sinus  venosus;  V.v.,  valvulse  venosse;  S.I.,  septum  primum.  Note  the 
bifid  apex  seen  at  this  stage  and  also  the  presence  of  two  openings  (O'  and  O")  in  the  primitive 
auricular  septum.  In  the  collection  of  the  I.  Anatomical  Institute,  Vienna.  (From  Tandler's  article  in 
Keibel  and  Mall's  Embryology,  vol.  ii,  p.  549.) 


The  septum  secundum  arises  considerably  later  than  the  septum  primum 
in  a  plane  a  little  to  its  right,  from  the  upper  wall  of  the  right  auricle,  and 
passes  downward  covering  in  the  upper  and  anterior  portion  of  the  ostium 
secundum,  thus  giving  it  a  valvular  character,  and  transforming  it  into  the 
foramen  ovale  of  fetal  life.  A  portion  of  the  septum  secundum  persists 
in  adult  life  as  the  aiumdus  ovalis,  while  the  valvula  foraminis  ovalis  of  the 
adult  left  auricle  represents  the  remains  of  the  primary  septum,  the  primary 
and  secondary  ostia  of  which  have  both  become  obliterated. 


CONGENITAL  CARDIAC  DISEASE  327 

The  Interventricular  Septum. — This  begins  about  the  fourth  week, 
just  after  the  origin  of  the  auricular  septum,  as  a  crescentic  ridge  on  the 
inferior  wall  of  the  ventricle.  It  grows  upward  and  backward,  its  pos- 
terior limb  merging  with  the  corresponding  walls  of  the  ventricle  and  with 
the  posterior  endocardial  cushion,  and  its  anterior  limb  with  the  antero- 
ventricular  wall  along  the  bulbo-atrial  ridges,  while  its  median  curved 
portion  unites  with  a  prolongation  of  the  proximal  aortic  septum  (the 
aortic  orifice  having  moved  over  from  the  right  to  over-ride  the  ventricular 
septum),  and  with  the  fused  endocardial  cushions  of  the  auriculo ventricu- 
lar orifice  (which  has  also  come  to  lie  in  the  median  line),  so  that  the  ven- 
tricles are  completely  separated  from  each  other  and  the  arterial  and 
venous  ostia  are  placed  one  in  either  ventricle  (Figs.  17  and  18).  The 
point  of  union  of  the  aortic  with  the  interventricular  septum  just  below  the 
adjacent  ends  of  the  anterior  and  left  posterior  aortic  cusps,  remains  trans- 
parent and  devoid  of  muscle  throughout  life,  and  is  known  as  the  pars 
membranacea,  or  undefended  space. 

The  Aortic  Septum. — The  truncus  arteriosiLS  is  divided  into  the  two 
great  efferent  vessels  of  the  heart  by  a  septum  derived  from  three  sources. 
Before  the  fifth  week  a  sharp  fold,  the  aortopulmonary  septum  proper, 
appears  in  the  lumen  of  the  truncus  at  the  point  of  junction  of  the  4th  and 
6th  arches  (which  represent  respectively  the  aortic  and  pulmonary  trunks) 
and  grows  rapidly  downward.  Some  distance  above  the  heart  this  aorto- 
pulmonary septum  proper  meets  and  fuses  with  a  spiral  septum  derived 
from  fusion  of  the  so-called  distal  and  proximal  endocardial  hidhar  swellings. 
The  bulbus  cordis  which  forms,  as  stated  above,  by  the  involution  of  its 
proximal  portion  the  termination  of  the  ventricle,  and  by  the  elongation 
and  demuscularization  of  its  distal  portion,  the  first  part  of  the  primitive 
aorta,  is  supplied  internally  with  a  series  of  endocardial  elevations,  of 
which  four  belong  to  its  distal  and  two  to  its  proximal  part  (known  respec- 
tively as  the  Distal  Bulbar  Swellings  1,  2,  3,  and  4,  and  Proximal  Bulbar 
Swellings  A  and  B  of  Born).  These  swellings,  while  symmetrically  placed 
on  opposite  sides  of  the  tube,  have  a  spiral  arrangement  from  above  down- 
ward, and  the  distal  swellings  2,  and  4,  which  are  much  more  prominent 
than  the  distal  swellings  1  and  3,  are  directly  continuous  in  clock-wise 
spiral  fashion  with  the  proximal  swellings  A  and  B.  Fusion  with  each 
other,  first  of  the  more  prominent  pair  of  the  distal  bulbar  swellings  and 
later  of  the  proximal  ones  occurs,  the  sjnral  bulbar  septum  resulting,  unit- 
ing at  its  distal  end  with  the  aortopulmonary  septum  proper,  and  the  two 
structures  being  clearly  distinguished  from  each  other  by  their  distinctive 
histological  characters. 

The  chambers  of  the  heart  and  the  two  great  arteries  have  thus  been 
completely  separated  from  each  other  before  the  eighth  week  of  fetal  life. 
Meantime,  the  right  horn  of  the  sinus  venosus  has  been  taken  up  in  the 
wall  of  the  right  auricle,  and  the  valvula  venosa  sinistra  has  disappeared, 
a  portion  of  the  valvula  venosa  dextra  persisting  as  the  Eustachian  valve, 
and  the  left  sinus  horn  remaining  as  the  coronary  sinus,  while  the  left  duct 
of  Cuvier  becomes  obliterated  (left  superior  vena  cava) .  The  pulmonary 
veins  form  later,  opening  at  first  as  a  single  trunk,  which  is  later  taken  up 
in  the  wall  of  the  left  auricle,  thereby  enlarging  it.     The  semilunar  cusps 


328 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


appear  to  form  about  the  seventh  week,  from  the  proximal  ends  of  the  four 
distal  bulbar  swellings,  two  of  which  are  subdivided  in  the  descent  of  the 
septum  trunci,  so  that  six  cusps,  three  placed  in  each  artery,  result. 

The  Auriculo ventricular  Cusps  and  the  Atrioventricular  Bundle  of  His. 
— The  most  critical  point  in  the  developing  heart  is  undoubtedly  the  atrial 
canal.  The  endocardial  cushions,  which  develop  within  it,  are  extensive 
and  vitally  important  structures,  not  only  as  taking  the  essential  part  in 
the  formation  of  the  venous  ostia,  but  also  as  completing  the  separation 
of  all  four  chambers  by  fusion  with  their  respective  septa.  Moreover, 
from  the  observations  of  Mall  on  a  large  series  of  early  human  embryos, 
we  learn  that  the  differentiation  of  the  auriculoventricular  bundle  of  His 


Fig.  20 


..2? 


Sagittal  section  through  the  heart  region  of  an  embryo  8  mm.  long,  X  40,  No.  113  of  Prof  Franklin 
P.  Alall's  collection.  B,  bulbus  cordis;  A.V.B.,  auriculoventricular  bundle;  A.F.,  annulus  fibrosis; 
I.V.C.,  interventricular  canal;  F.O'.,  ostium  primum;  P.O"..  ostium  secundum;  S.I.,  septum  primum; 
E-,  endocardial  cushions.  Note  the  extensive  development  of  the  endocardial  cushions  -within  the  auricle. 
(From  the  article  by  F.  P.  Mail  in  the  American  Journal  of  Anatomy,  July  15,  1912.) 

is  to  be  traced  to  the  breaking  of  the  continuity  of  the  atrial  with  the  ven- 
tricular musculature  by  the  ingrowth  of  constricting  epicardial  connective 
tissue  about  the  external  surface  of  the  atrial  ring.  Thus  while  in  very 
early  stages  the  muscle  of  the  auricle  is  continuous  with  that  of  the  ven- 
tricle at  all  points,  in  later  stages  a  single  band  of  atrial  tissue  passing 
down  posteriorly  from  the  lower  border  of  the  sinus  venosus  to  the  ven- 
tricle, and  two  minor  fasciculi  on  the  anterolateral  wall,  are  the  only 
remaining  connection  between  the  chambers.  The  survival  of  these 
isolated  portions  in  the  general  destruction  of  the  muscular  continuity 
between  auricle  and  ventricle  is  explained  by  Mall  by  the  anatomical  rela- 
tions of  the  posterior  part  of  the  interventricular  septum,  which,  growing 


CONGENITAL  CARDIAC  DISEASE  329 

up  toward  the  posterior  endocardial  cushion,  pushes  the  epicardial  tissue 
obHquely  before  it  and  permits  the  escape  of  a  small  portion  of  auricular 
muscle.  This  surviving  atrial  tissue,  now  the  only  path  of  conductivity, 
undergoes  differentiation  and  later,  innervation,  and  becomes  readily 
identified  as  the  auriculoventricular  bundle. 

This  contention  of  !Mall,  that  the  bundle  represents  persistent  atrial 
tissue,  and  that  its  survival  at  this  point  is  the  result  of  its  anatomical 
relation  with  the  interventricular  septum,  has  received  striking  confirma- 
tion from  the  investigations  of  Monckeberg,  and  Sato^  in  the  distribution 
of  the  bundle  in  various  cardiac  defects.-  Thus  in  a  Cor  Triloculare  Bia- 
triatrmn  where,  in  the  entire  absence  of  ventricular  septum  one  might  con- 
clude a  destruction  of  auricular  tissue  in  the  whole  circumference  of  the 
atrial  ring,  this  bundle  was  found  to  be  absent  from  the  normal  situation 
and  was  represented  by  a  small  band  of  tissue  accompanying  a  small  vessel 
on  the  anterolateral  aspect  of  the  heart.  In  defects  of  the  interventricu- 
lar septum  at  the  base  on  the  other  hand,  in  which  the  posterior  part  of 
the  septum  practically  always  remains  entire,  the  bundle  was  seen 
intact  in  both  ventricles,  streaming  over  the  lower  border  of  the  defect. 

The  auriculoventricular  cusps  are  formed  from  the  endocardial  cushions 
of  the  atrial  canal.  These  cushions  are  a  series  of  elevations  of  the  lining 
endothelium  of  the  cardiac  tube  formed  by  a  spongy  connective  tissue, 
and  are  four  in  number.  Two,  the  anterior  and  the  posterior,  develop  very 
early,  become  of  large  size,  and,  growing  toward  each  other,  fuse  to  form 
the  wedge-shaped  block  which  separates  the  venous  ostia  and  completes 
the  cardiac  septa.  In  addition  they  encroach  by  their  rapid  growth  on 
adjacent  structures,  so  that  they  come  to  line  the  lower  border  of  the 
septum  primum  in  the  auricle,  while  extending  also  by  their  apices  into  the 
depths  of  the  ventricle.  The  lateral  cushions,  of  smaller  size,  develop 
later,  and,  with  the  anteroposterior  pair,  are  converted  from  endocardial 
structures  into  the  musculotendinous  valves,  by  the  undermining  of  their 
substance  from  without,  and  by  their  own  invasion  of,  and  fusion  with,  the 
spongy  musculature  of  the  ventricle. 

Primitive  Aortic  Arches. — After  considerable  discussion,  it  is  now  fairly 
demonstrated  that  these  number  six  instead  of  five,  as  Rathke  described, 
the  disputed  fifth  arch  being  rudimentary  in  character.  They  are  more  or 
less  evanescent  in  all  animals,  except  fishes,  in  which  five  persist.  In  birds 
and  mammals  the  first,  third,  and  fifth  disappear  on  both  sides.  In  man 
the  fourth  left  arch  becomes  the  aorta,  the  fourth  right,  the  right  sub- 
clavian, while  the  sixth  pair  become  the  pulmonary  arteries. 

LITERATURE. 

The  rarity  of  cardiac  defects,  the  obscurity  of  their  etiology  and  symp- 
toms, together  with  the  fact  that  the  cases  are  often  of  serious  clinical 
import,  make  the  subject  of  congenital  cardiac  disease  of  the  highest 

1  Aschoff,  Ber.  d.  Naturfor.  Gesell.  zu  Freiburg,  December  3,  191.3,  B.  xx. 

2  Professor  Adami  informs  me  that  this  idea  of  the  persistence  of  the  primitive 
atrial  tissue  in  the  Purkinje  fibres  of  the  adult  heart  was  suggested  by  Gaskell  in  his 
book  on  the  Evolntion  of  the  Vertebrates  (1904). 


330  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

interest.  Since  the  time  of  Senac^  it  has  attracted  the  interest  of  many  of 
the  ablest  workers  in  the  field  of  cardiac  pathology.  There  are  important 
special  contributions  from  nearly  all  the  earlier  writers  upon  the  heart, 
including  Morgagni,  Wm.  Hunter,^  Meckel,^  Louis/  Farre/  Breschet, 
Sir  James  Paget/  Gintrac/  Chevers/  and  Rokitansky.^  The  first  com- 
prehensive study  of  the  whole  subject  with  a  review  of  this  earlier  litera- 
ture, may  be  said  to  be  Peacock's,^"  which  remains  a  classic  and  is  still  the 
leading  authority  in  English  upon  the  subject.  In  Germany  the  ground 
has  been  covered  by  Lebert-Schrotter^^  (1879),  by  Rauchfuss^^  (1878), 
by  Vierordt^^  (1898),  in  a  statistical  study  of  great  value,  and  more  recently 
by  Thoreli^  (1903  and  1911),  and  Herxheimeri^  (1910).  In  English  there 
are  the  excellent  general  accounts  of  Humphry,^''  Carpenter,^^  and 
Keith,^^  and  in  French  the  work  of  Moussous,^^  Gerard,^'^  and  Theremin,^^ 
The  last  is  a  study  of  much  value  comprising  106  observations  of  cardiac 
defects  with  measurements  and  illustrative  plates. 

Perhaps  the  most  valuable  and  certainly  the  most  brilliant  original 
contribution  has  been  Rokitansky's.  This  is  an  analysis  of  44  cases  of 
complicated  septal  defects,  together  with  a  study  of  the  normal  anatomy 
of  the  septa,  and  of  their  development  as  observed  by  Rokitansky  himself, 
in  the  human  embryo  and  in  the  chick.  Rokitansky  explained  all  cardiac 
anomalies  associated  with  septal  defects  as  due  to  arrest  in  the  develop- 
ment of  the  cardiac  or  aortic  septa,  and  to  their  consequent  non-union  or 
irregular  union.  Although  the  results  of  his  observations  have  been  modi- 
fied, his  work  is  of  inestimable  value  as  giving  a  clue  to  many  problems, 
and  especially  in  regard  to  transposition  of  the  arterial  trunks. 

Reference  has  already  been  made  to  the  recent  work  of  Keith. 
From  a  series  of  personal  observations  on  conus  stenosis,  and  from  the 
study  of  270  malformed  hearts  in  the  museums  of  London,  he  has  advanced 
the  view  that  cardiac  defects  are  nearly  always  developmental  in  origin, 
that  pulmonary  stenosis  is  usually  due  to  subinvolution  of  the  bulbus 
cordis  and  that  transposition  results  from  an  irregularity  in  involution  of 
the  same  primitive  structure. 

1  Traite  de  la  structure  du  coeur,  de  son  action  et  de  ses  maladies,  Paris,  1749, 

2  Medical  Observations  and  Enquiries,  London,  1784,  vol.  vi,  p.  291. 

3  De  Cordis  conditionibus  abnormibus.  Dissertation,  Halle,  1802. 
^  Memoir es  ou  recherches  anatomico-pathologiqries,  Paris,  1826. 

^  On  Malformations  of  the  Human  Heart,  London,  1814. 

6  Edinburgh  Medical  and  Surgical  Journal,  1831,  vol.  xxxvi,  p.  263. 

^  Recherches  sur  la  maladie  bleue,  Paris,  1824. 

^  London  Medical  Gazette,  series  of  articles,  1845  to  1851. 

9  Defekte  der  Scheidewdnde  des  Herzens,  Vienna,  1875. 

Malformations  of  the  Human  Heart,  1858  and  1866. 

Article  in  Ziemssen's  Handbuch  der  spec.  Path,  et  Ther.,  Leipsic,  1879,  Band  vi. 

In  Gerhardt's  Handb.  d.  Kinder krankheiten,  1878,  iv,  part  i. 

NothnageVs  Spec.  Path.  u.  Therapie,  Bd.  xv,  1898.     Tli.  1.,  Abt.  11. 
*  Lubarsch  and  Ostertag' s  Ergebnisse,  1  Abth.,  1903,  p.  585,  and  11  Abth.,  1910,  p.  268. 
^  Schwalbe's  Missbildungen,  iii  Th.,  iii  Lief,  2  Abth.,  1910. 
^  Allbult's  System  of  Medicine,  vol.  iv. 
->  Brit.  Jour.  Child.  Dis.,  1909,  vol.  vi,  pp.  337,  385,  433. 
8  Lancet,  1909,  vol.  ii,  359,  433,  519. 
8  Encyclop.  Scient.  des  aide-memoires,  Paris. 

2"  Rev.  de  med.,  1900,  pp.  645  and  837;  also  Jour,  de  I'Anat.,  1900,  pp.  1  and  323. 
21  Etudes  sur  les  affections  congenitales  du  coeur,  Paris,  1895. 


CONGENITAL  CARDIAC  DISEASE  331 

The  bulk  of  the  hterature  centres  about  three  questions,  which  may 
be  stated,  with  the  theories  promulgated  upon  them,  as  follows : 

1.  The  cause  of  the  defect.  Is  it  developmental  or  due  to  intra-uterine 
disease? 

2.  The  causation  of  the  cyanosis  so  often  present :  Is  it  due  to  admixture 
of  venous  and  arterial  currents;  to  delayed  aeration  of  blood;  to  both  these 
conditions,  or  to  still  obscurer  causes  associated  with  changes  in  tissue 
metabolism  and  in  the  composition  of  the  blood  itself? 

3.  In  the  combination  so  frequently  occurring  of  defect  of  the  inter- 
ventricular septum  with  stenosis  of  the  pulmonary  artery,  is  the  septal 
defect  secondary,  due  to  the  rise  of  pressure  behind  the  stenosed  orifice 
before  closure  of  the  fetal  passages  had  occurred;  or  is  it  primary,  the 
deflection  of  the  current  of  blood  through  the  defect  leading  to  hypoplasia 
of  the  pulmonary  artery  through  disuse  ?  Or  are  both  conditions  the  result 
of  a  common  cause,  an  arrest  or  deviation  or  other  irregularity  in  develop- 
ment? 

An  analysis  has  been  made  of  the  records  of  631  cases  of  congenital  car- 
diac disease  which  serve  here  as  an  illustrative  basis.  Of  these,  205  have 
been  drawn  from  the  Trajisactions  of  the  Pathological  Society  of  London, 
a  few  from  personal  experience,  and  the  remainder  from  the  literature. 
English  and  American  records  have  been  consulted  so  far  as  possible,  as 
these  sources  are  often  overlooked  in  previous  statistical  studies,  which 
are  largely  Continental. 

ETIOLOGY  OF  CONGENITAL  CARDIAC  DISEASE. 

Cardiac  anomalies  may  be  divided,  according  to  etiology,  into  two 
main  groups:  those  due  to  arrest  of  growth  at  an  early  stage,  before 
the  different  parts  of  the  heart  have  been  entirely  formed,  and  those 
produced  in  the  more  fully  developed  heart  by  fetal  disease. 

Arrest  of  Growth. — From  the  earliest  times  search  has  been  made 
for  the  underlying  causes  of  the  arrest  of  development  manifest  in  cardiac 
malformations.  Long  before  Darwin,  Meckel,  in  1812,  pointed  out  the 
resemblance  of  certain  defects  to  the  hearts  of  those  animals,  which 
present  in  a  stationary  form  the  different  stages  through  which  the 
mammalian  heart  passes  in  its  development,  and  explained  them  as 
reversions  to  a  more  primitive  type. 

In  seeking  the  causes  of  the  defect  we  may  turn  first  to  the  study  of 
associated  anomalies.  Do  these  occur  in  such  frequency  and  constancy 
as  to  place  their  combination  beyond  the  range  of  coincidence?  And  if 
so,  may  the  causes  leading  to  malformations  elsewhere,  such  as  disease 
and  adhesions  of  the  amnion,  maternal  disease,  hereditary  predisposition, 
etc.,  be  assumed  to  act  upon  the  fetal  heart? 

In  Rokitansky's  Defekie  der  Scheidewdnde  des  Herzens,  among  24 
complicated  defects  of  the  septum,  all  evidently  of  developmental  origin, 
associated  anomalies  such  as  transposition  of  the  viscera,  cleft  palate, 
etc.,  occurred  in  8,  that  is,  in  one-third  of  the  cases.  Yierordt,  in  the 
700  cases  reviewed  by  him,  found  associated  anomalies  in  80  (11  per 
cent.).     On  the  other  hand  Keith  found  among  23  malformed  fetuses 


332  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

and  infants  showing  anencephaly,  hydrocephaly,  spina  bifida,  umbiHcal 
hernia,  atresia  ani,  cleft  palate,  harelip,  and  stricture  of  the  oesophagus, 
in  14  a  malformation  of  the  heart. 

Among  the  631  cases  studied  here,  anomalies  elsewhere  in  the  body, 
among  which  may  be  enumerated  malformations  of  liver  and  lung, 
asymmetry  of  calvarium,  partial  or  complete  transposition  of  viscera, 
harelip  and  cleft  palate,  encephalocele,  gastro-  and  rachischisis,  absence 
of  spleen  or  kidney,  diverticula,  h^^pospadias,  hernia,  etc.,  occurred  in 
92  cases,  that  is,  in  14  per  cent.  Defect  of  the  interventricular  septum 
was  associated  in  Chaffey's  case  with  imperforate  anus,  in  Moore's 
with  a  supernumerary  thumb,  in  Morestin's  with  syndactylism  and 
absence  of  femur,  fibula,  and  genitalia.  A  widely  patent  foramen  ovale 
was  combined  in  Berthel's  case  with  rudimentary  genitalia,  and  in 
Tylecote's  with  a  congenital  perforation  of  the  nasal  septum.  Kingsley 
reports  patent  ductus  with  macroglossia  and  absence  of  the  left  kidney, 
and  Dick  a  case  of  pulmonary  artery  forming  the  descending  aorta,  with 
the  uterus  bipartite,  and  the  kidneys  fused.  Mental  deficiency  or  de- 
rangement of  the  higher  nerve  centres  is  not  infrequent.  Thus  idiocy 
was  reported  by  Simmons,  Carpenter  and  Rheiner,  in  cases  of  patent 
foramen  ovale,  patent  ductus,  and  septal  defect  respectively,  and  in  a 
biloculate  heart  reported  by  Dublizhaza,  idiocy  was  combined  with 
strabismus  and  pes  varus. 

Further  illustrations  might  be  multiplied,  but  the  above  suffice  to 
show  that  the  association  of  grave  anomalies  with  cardiac  defects  is  too/ 
frequent  to  be  considered  accidental.  That  the  cause  of  both  is  to  be 
sought,  not  so  much  in  a  hereditary  predisposition,  as  in  a  diseased 
condition  of  the  fetal  envelopes  or  of  the  maternal  tissues  is  evident  from 
the  facts  yielded  by  the  family  history  of  these  cases.  For  a  history 
]of  congenital  disease  in  the  ancestry  is  much  less  common,  than  is  one 
of  cardiac  defect  or  other  anomaly  in  other  members  of  the  same  gener- 
ation, and  evidence  of  infective  processes  or  depressing  influences  acting 
within  the  parental  organism  is  still  more  frequently  supplied.  In  this 
series  there  was  a  history  of  congenital  defect  in  a  brother  or  sister  of  the 
patient  in  11  cases,  of  rheumatism  or  heart-disease  in  the  parents  in 
13,  and  of  small-pox  or  tuberculosis  in  six.  Congenital  syphilis  in  the 
father  was  recorded  by  Jacobi,  in  a  case  of  ectopia  cordis,  and  lues 
whether  congenital  or  acquired  is  certainly  a  frequent  cause.  Baneful 
influences  acting  upon  the  mother  during  the  early  weeks  of  pregnancy 
have  been  described,  such  as  great  trouble,  ill-treatment  and  fright. 
Severe  inflammation  of  the  bladder  of  the  mother  in  the  third  month 
was  blamed  by  Habershon  for  the  development  of  pulmonary  atresia 
with  septal  defect,  and  an  operation  on  the  mother  for  appendicitis  was 
noted  by  Royer  and  Wilson,  in  their  case  of  incomplete  heterotaxy. 
In  not  a  few  instances  the  parents  had  both  reached  advanced  middle 
life,  and  in  some  the  child  was  the  last  of  a  series  of  many  pregnancies. 

Difficult  delivery  occurred  in  the  cases  of  patent  ductus  by  Luys, 
Roeder,  and  others.  Laine  reported  a  case  of  aortic  stenosis  with  septal 
defect  from  a  mother  aged  forty-eight  years,  who  had  had  four  other 
children,  of  whom  three  were  feeble-minded. 


CONGENITAL  CARDIAC  DISEASE  333 

The  predominating  cause  of  the  defect  is  thus  clearly  to  be  sought  in 
the  immediate  environment  of  the  developing  embryo.  It  must  be  rec- 
ognized that  the  early  death  in  most  cases  of  congenital  cardiac  disease 
prevents  direct  transmission  of  cardiac  defects,  which  might  otherwise 
occur,  and  this  lessens  the  apparent  frequency  of  heredity.  That 
heredity  is  a  factor  in  a  certain  proportion  of  cases  is  evidenced  by 
^numerous  facts.  The  association  of  symmetrical  polydactylism  is  sig- 
nificant when  one  considers  the  well  known  familial  tendency  of  this 
anomaly.  Of  much  interest  also  is  a  specimen  reported  by  J.  McCrae^ 
in  which  transposition  of  the  viscera  and  atresia  of  the  pulmonary 
artery  were  found  in  the  fifteenth  child  of  a  forty-six-year-old  mother, 
who  was  herself  of  poor  intelligence  and  had  a  harelip. 

Fetal  Disease. — Acute  endocarditis  w^as  formerly  claimed  to  be  a 
widely  acting  cause  of  congenital  cardiac  disease,  not  only  in  those 
instances  in  which  in  the  fully  formed  heart  the  traces  of  its  presence  are 
incontestable,  but  in  the  earlier  cases  of  arrest  of  growth  which  were 
explained  as  due  to  its  action  upon  the  half-developed  embryo.  With 
the  increasing  knowledge  of  development,  the  trend  of  modern  opinion 
is  to  explain  the  majority  of  cardiac  defects  as  arrest  of  gro  w^th,  dependent 
upon  a  variety  of  causes  as  enumerated  above,  and  to  class  with  these 
many  cases  of  pulmonary  stenosis  and  atresia,  formerly  thought  to  be 
inflammatory  in  origin.  There  remains  a  certain  proportion  due  to  fetal 
endocarditis,  but  it  is  impossible  to  state  the  exact  degree  of  its  influence, 
and  it  is  therefore  safest  to  divide  cardiac  defects  into  two  classes,  viz., 
those  in  which  an  arrest  of  development  from  any  cause  has  taken  place,  I 
and  those  which  show,  by  thickening  and  cicatricial  contraction,  that 
they  have  been  produced  by  fetal  disease  after  the  heart  has  been  fully! 
formed. 

The  presence  of  thickening  of  the  endocardium  does  not  prove  that  a 
defect  is  originally  due  to  an  inflammatory  process,  for  cardiac  defects, 
giving  rise  so  readily  to  abnormal  currents  and  to  undue  strain  upon  the 
valves,  are  particularly  liable  to  be  the  seat  of  future  disease. 

CYANOSIS. 

Congenital  cyanosis  is  a  bluish  discoloration  of  the  skin  and  mucous 
membranes,  characterizing  the  more  pronounced  cases  of  congenital 
cardiac  disease  in  which  there  is  serious  interference  with  the  circulation. 
It  differs  from  the  cyanosis  of  the  later  stages  of  acquired  cardiac  lesions 
in  that  it  may  exist  for  many  years  w^ithout  any  signs  of  cardiac  insuffi- 
ciency. Its  constant  association  with  the  other  evidences  of  deficient 
oxygenation — dyspnoea  and  clubbing— raises  it  almost  to  the  ranks  of  a 
disease  entity,  and  such,  under  the  titles  Cyanopathia  or  Morbus  Cceruleus, 
it  was  long  believed  to  be. 

Pathogenesis. — The  immediate  causation  has  long  been  the  subject 
of  debate.  It  has  been  variously  ascribed  to  (a)  venous  stasis,  (h)  ad- 
mixture of  currents,  (c)  deficient  aeration,  {d)  dilatation  and  new  for- 

1  Jour.  Anat.  and  Physiol.,  1905,  xl,  48. 


334  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

mation  of  capillaries  in  peripheral  parts  of  the  body,  (e)  changes  in  the 
blood  itself,  e.  g.,  polycythemia.  The  last  two  conditions,  being  sub- 
ordinate factors  and  secondary  causes  only,  may  be  dismissed. 

(a)  The  term  venous  stasis  is  used  in  this  connection  to  imply  obstruc- 
tion to  the  free  entrance  of  the  blood  to  the  lungs  and  the  resultant  back- 
pressure in  the  systemic  circulation.  This  theory,  advanced  by  Morgagni, 
has  been  largely  accepted,  but  does  not  explain  the  situation  completely. 
It  is  difficult  to  understand  why  a  simple  venous  stasis  should  be  sufficient 
to  lead  to  cyanosis  and  yet  remain  unassociated  with  the  oedema  and 
anasarca  accompanying  back-pressure  from  other  causes.  And,  on  the 
other  hand,  the  late  appearance  of  the  cyanosis  in  many  cases  of  pulmo- 
nary stenosis  in  which,  although  the  defect  has  been  undoubtedly  present 
at  birth,  cyanosis  only  supervenes  after  some  months  or  years  on  the 
occurrence  of  some  event  temporarily  increasing  the  embarrassment  of 
the  pulmonary  circulation  renders  it  evident  that  some  other  factor,  in 
addition  to  the  mechanical  difficulties  which  the  lesion  presents,  is,  as  a 
rule,  needed  to  bring  it  about.  A  highly  instructive  case  is  published  by 
Lafitte^  of  a  young  woman,  dying  at  twenty-one  years  of  a  malignant 
endocarditis,  who  had  always  been  dyspnoeic  on  slight  exertion  but  had 
never  presented  any  trace  of  cyanosis.  At  the  autopsy  the  right  heart 
was  hypertrophied  and  about  one  inch  below  the  pulmonary  valves 
there  was  a  fibrous  annular  stenosis  of  the  infundibular  orifice  which  was 
further  blocked  by  large  recent  vegetations.  Peacock,  in  reporting  a 
case  of  pulmonary  stenosis  without  cyanosis,  suggested  that  the  absence 
of  s}'mptoms  was  due  to  the  marked  hypertrophy  of  the  right  ventricle 
which  had  succeeded  in  sending  sufficient  blood  to  the  lungs  for  aeration. 

(6)  The  theory  that  cyanosis  is  due  to  a  mingling  of  venous  with  arterial 
blood  (wrongly  ascribed  to  Hunter,  as  Osier  points  out)  has  been  sharply 
and  apparently  successfully  refuted  by  many  authorities,  notably  Pea- 
cock. Certain  strong  arguments  can  certainly  be  adduced  against  its 
universal  application.  The  classical  illustration  is  Breschet's  case,  in 
which  the  left  subclavian  arose  from  the  pulmonary  artery,  and  yet 
the  left  arm  was  normal,  not  discolored.  Again,  in  many  instances  of 
biloculate  or  triloculate  heart  there  is  a  complete  absence  of  cyanosis. 
Thus  Young-  reports  a  cor  biatriatum  triloculare,  both  auricles  opening 
into  a  common  ventricle,  from  which  arose  the  aorta  and  pulmonary 
artery,  transposed  and  separated  from  each  other  by  an  anomalous  septum 
in  a  man,  aged  thirty-six  years,  who  showed  no  cyanosis  until  the  last 
three  years  of  life.  Peacock  quotes  an  almost  identical  case  in  an  infant 
aged  eight  months,  with  only  a  slight  blueness  of  the  lips  during  dyspnoeic 
attacks.  Equally  striking  is  a  case  of  persistent  truncus  arteriosus,  in 
which,  although  the  blood  from  both  ventricles  entered  the  common 
arterial  trunk,  cyanosis  was  absent. 

The  theory  of  admixture  of  currents  has  been  revived  by  Bard  and 
Curtillet^  in  a  form  that  has  been  generally  accepted.  They  describe 
as  cyanose  tardive  a  cyanosis  occurring  as  a  terminal  event,  often  at 

1  Bull,  de  la  Soc.  Anal.,  1892,  vi,  13. 

-  Med.  Chron.,  Manchester,  1907-08,  xiv,  96. 

^  Rev.  de  med.,  December,  1889. 


CONGENITAL  CARDIAC  DISEASE  335 

the  end  of  a  long  life,  in  cases  of  patent  foramen  ovale,  when  some 
embarrassment  in  the  pulmonary  circulation  causes  a  raised  pressure 
in  the  right  heart  leading  to  a  flow  of  blood  through  the  foramen  and 
sometimes  to  a  forced  reopening  when  it  has  been  closed.  They  quote 
an  illustration  in  a  man  aged  fifty-four  years,  with  patent  foramen, 
dying  of  bronchopneumonia.  Long  before  this.  Peacock^  reported  such 
a  case,  in  a  woman,  aged  twenty-four  years,  with  marked  spinal  curvature 
and  widely  patent  foramen  ovale,  in  whom  marked  cyanosis  set  in  for 
the  first  time  in  the  last  months  of  life. 

That  cyanosis  may  occur  without  admixture  of  currents,  in  the  ordinary 
application  of  the  term,  is  definitely  shown:  (1)  by  its  presence  in  a 
limited  number  of  cases  of  congenital  pulmonary  stenosis,  in  which  the 
fetal  passage's  are  all  closed,  and  (2)  by  the  fact  that  the  most  marked 
pictures  of  cyanosis  with  clubbing  may  occur  in  acquired  pulmonary 
emphysema  and  in  bronchiectasis.  But  in  these  latter  combinations 
lies  perhaps  the  key  to  the  situation.  In  bronchiectasis,  as  Thomas^ 
points  out,  areas  of  loss  of  substance  occur,  and  tortuous  dilated  capillaries 
with  thickened  walls  exist,  and  it  is  readily  conceived  that  in  certain 
areas  blood  may  pass  from  pulmonary  arterioles  to  venules  without 
undergoing  due  oxygenation  by  the  way.  In  congenital  cardiac  disease 
dilatation  and  thickening  of  peripheral  vessels  form  a  part  of  the  picture, 
and  alterations  very  similar  to  those  observed  in  bronchiectasis  occur. 
In  a  case  described  by  Carpenter^  the  lungs  were  loaded  with  pigment, 
their  capillaries  dilated  to  three  times  their  normal  size,  crowded  with 
red  cells,  elongated,  tortuous,  their  walls  thickened  and  rich  in  young 
fibrous  tissue  elements.  Must  not  many  red  cells  have  passed  through 
these  thickened  channels  without  receiving  their  due  share  of  oxygen, 
and  the  blood  have  been  thus  returned,  still  largely  venous  in  character, 
to  the  left  heart.  Viewed  in  this  light  do  not  venous  stasis  and  admixture 
of  currents  become  only  a  distinction  in  terms  between  two  conditions 
leading  alike  to  deficient  aeration? 

(c)  Changes  in  the  Bloodvessels  and  Tissues. — It  was  suggested  by 
Carpenter  that  the  changes  produced  in  the  lungs  by  the  circulation  of 
venous  blood  might  lead  to  the  cyanosis  by  creating  pulmonary  obstruc- 
tion. Certainly  a  vicious  circle  is  created  but  the  altered  capillary  cir- 
culation, being  itself  the  result  of  deficient  aeration,  can  be  looked  upon 
only  as  a  secondary  cause  of  any  symptoms  it  may  help  to  produce. 

{d)  Changes  in  the  Blood  Itself. — ^The  dark  color  of  cyanotic  blood 
has  been  ascribed  to  the  great  increase  in  red  blood  corpuscles  which 
often  exists.  The  polycythemia,  however,  can  have  no  causal  relation 
to  the  cyanosis,  for  it  not  only  is  not  constant  in  the  congenital  form, 
but  a  very  high  blood  count  is  consistent  with  an  entire  absence  of 
cyanosis,  as  is  evidenced  in  the  polycythemia  of  high  altitudes. 

(e)  The  theory  that  a  variety  of  causes,  including  both  mingling  of 
currents,  venous  stasis  and  pulmonary  obstruction,  lead  to  a  deficient 
aeration  of  the  blood  and  that  this  is  the  essential  element  in  the  production 

1  Trans.  Path.  Soc,  London,  1859,  x,  108. 

2  Zeit.  f.  klin.  Med.,  1901,  xli,  58. 

5  St.  Thomas  Hospital  Reports,  1890,  xviii,  285. 


336  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

of  cyanosis,  is  formulated  by  many  recent  observers.  There  is  abundant 
evidence  to  show  that  whatever  the  path  by  which  oxygenation  is  reduced, 
whether  by  direct  influx  of  venous  blood  into  the  arterial  tree,  or  by 
obstruction  to  the  entrance  of  venous  blood  into  the  pulmonary  circulation 
the  deficient  aeration  resulting  is  in  all  cases  of  congenital  cyanosis  the 
immediate  cause  of  the  symptomatology.  The  characteristic  picture 
can  be  traced  through  the  development  of  the  compensatory  mechanism 
of  right  heart  hypertrophy  and  increased  respiratory  activity,  and  poly- 
cythemia, to  the  point  where  all  these  processes  fail  to  supplement  in 
the  inefficient  pulmonary  circulation  with  oxygen  sufficient  for  the  body 
needs.  The  chronic  asphyxia  that  develops  is  expressed,  not  only  in  the 
cyanotic  hue  of  the  patient,  but  also  in  the  alterations  at  the  periphery 
of  clubbing  and  retinal  changes.  These  are  the  direct  results  of  delayed 
and  toxic  tissue  metabolism,^  and  an  overloaded  systemic  circulation. 

The  above  considerations  may  be  summarized  by  saying  that  the 
dependence  of  cyanosis,  with  its  attendant  phenomena,  upon  deficient 
oxygenation  may  be  accepted  as  a  fact;  that  the  circulation  is  evidently 
able  to  accommodate  itself  to  a  certain  degree  of  de-oxygenation,  whether 
this  be  brought  about  by  obstruction  in  the  course  of  the  pulmonary 
artery,  by  a  general  retardation  of  flow,  or  by  a  mingling  of  venous  with 
arterial  blood,  but  that  as  soon  as  deficient  hematosis  reaches  a  certain 
limit,  oxygenation  becomes  insufficient  for  the  needs  of  the  body,  and 
cyanosis  results.  Pulmonary  obstruction  alone  appears  capable  of  pro- 
ducing cyanosis,  but  it  is  still  a  question  as  to  which  of  the  above  factors 
is  the  essential  one,  or  in  what  degree  they  must  be  combined  to  bring  the 
circulation  to  this  limit,  or  what  is  the  amount  of  venous  blood  which 
can  circulate  without  producing  symptoms  of  deficient  aeration.  That 
dilated  peripheral  capillaries,  dark  color  and  increased  red  cell  content 
of  the  blood,  must,  when  present,  add  their  part  to  heighten  the  degree 
of  discoloration  is  self-evident;  but  being  themselves  secondary,  these 
conditions  are  not  to  be  looked  upon  as  etiological  factors,  but  rather 
as  concomitant  effects  of  a  common  cause.  Lastly,  in  complicated  cardiac 
defects  probably  all  the  factors  enumerated  combine  to  produce  the 
mulberry  hue  and  the  respiratory  distress  of  the  typical  morbus  cser ulcus. 

Symptoms. — The  degree  of  discoloration  varies  from  a  slight  bluish 
tinge  of  the  cheeks„and  mucous  .membran^  appearing  on  exertion  or 
excitement,  to  a  distinctly  leaden  hue  of  the  whole  surface,  becoming 
purple  in  extreme  cases.  It  usually  increases  gradually,  and  in  many 
cases  marked  at  the  last  it  is  absent  at  birth,  appearing  after  weeks, 
months,  or  even  years,  when  some  intercurrent  event  has  heightened 
the  embarrassment  in  the  pulmonary  circulation.  As  a  general  rule,  to 
which  there  are  a  good  many  exceptions,  the  degree  of  cyanosis  may  be 
said  to  depend  upon  the  character  of  the  defect.  Thus  it  is  usually  slight 
or  even  entirely  absent,  except  during  dyspnoeic  attacks,  or  as  a  terminal 
event,  in  patent  foramen  ovale,  patent  ductus,  or  septal  defects;  a  quite 
moderate  degree  characterizes  those  anomalies,  such  as  hiloculate  or 
trilocidate  heart,  in  which  there  is  a  free  intermingling  of  the  two  blood 

1  Deut.  Arch.  f.  klin.  Med.,  1910,  xcix,  382. 


CONGENITAL  CARDIAC  DISEASE  337 

streams,  but  no  pulmonary  obstruction;  the  marked  cases  such  as  are 
evident  to  every  casual  observer  as  "congenital  heart  disease,"  occur 
nearly  always  in  'pulmonary  stenosis  with  or  without  septal  defect;  and 
the  most  extreme  grades — the  typical  "blue  baby,"  are  seen  in  the  com- 
plete interference  with  the  blood  supply  to  the  lungs  which  takes  place 
in  pidmonary  atresia  or  transposition  of  the  great  trunks. 

The  subject  of  advanced  congenital  cyanosis  presents  a  striking 
appearance.  The  superficial  vessels  are  often  dilated,  the  face  congested, 
the  tongue  "geographical,"  the  eyes  discolored,  and  sometimes  bulging, 
the  tips  of  the  fingers,  toes,  and  nose,  flattened  and  bulbous,  and  the 
respirations  heightened  to  actual  dyspnoea.  Traces  of  anasarca  and 
oedema  sometimes  occur,  but  form  no  essential  part  of  the  picture, 
although  of  course  present  at  the  close  in  cases  which  terminate  with 
failing  compensation.  The  temperature  is  usually  low,  especially  in  the 
extremities,  and  there  is  a  tendency  to  catarrh,  severe  coughs,  and  colds 
on  slight  provocation.  Hemorrhages,  especially  from  the  nose,  and 
spitting  of  blood  are  prone  to  occur.  Disturbances  of  eyesight,  dimness 
of  vision  or  even  blindness,  may  occur  from  neuroretinitis. 

Delayed  development  is  frequent.  In  Goodman's^  case,  patent  ductus 
arteriosus  in  a  boy  aged  fourteen,  this  was  evidenced  by  retarded  ossifica- 
tion of  the  pisiform  bone  of  the  wrist  on  a'-ray  examination  {Rotch's  sign). 
The  patients  are  sometimes  of  very  high  intelligence,  but  when  the  cyano- 
sis has  set  in  early  they  are  often  stunted  mentally  as  well  as  physically, 
somnolent  in  thought,  and  sluggish  in  action.  In  females  the  menstrual 
function  is  often  delayed  in  onset,  scanty,  and  irregular.  In  the  cases  with 
marked  polycythemia,  in  addition  to  peripheral  signs  of  venous  congestion 
and  plethora,  cyclic  (postural  or  orthostatic)  albuminuria  may  occur. 
Parkes  Weber  records  a  case  in  a  youth  aged  twenty-two,  with  great 
cyanosis  and  clubbing  and  a  blood-count  of  10,300,000  red  cells,  in  whom 
repeated  examination  showed  the  early  morning  urine  to  be  free  from 
albumin,  while  that  passed  at  11  a.m.,  contained  a  considerable  amount. 
Cyanotic  patients  bear  the  acute  infections  of  childhood  well,  but  fre- 
quently succumb  to  pulmonary  tuberculosis.  Another  common  cause  of 
death  is  broncho-pneumonia. 

Special  Symptoms. — Clubbing  and  Cyanosis  Retinas. — These  symptoms 
constitute  the  visible  evidences  of  a  generalized  dilatation  of  the  smaller 
bloodvessels  over  the  body  surface  with  accompanying  productive 
changes.  New  formed  capillaries,  arterioles  with  thickened  walls,  tortuous 
dilated  veins,  and  new  connective  tissue  formation  have  been  observed 
in  advanced  cyanosis,  both  in  the  skin  (Variot  and  Gampert^)  and  in  the 
lungs,  as  well  as  in  the  retina  and  bulbous  finger  ends.  The  causation 
of  these  vascular  and  tissue  changes  is  certainly  complex  but  their  chief 
source  is,  undoubtedly,  stasis  and  lack  of  oxygenated  blood,  and  the 
effect  that  these  conditions  produce  upon  the  tissues,  by  means  of  the 
toxic  products  of  metabolism  which  escape  oxygenation. 

In  clubbing  of  the  extremities  especially,  these  mechanotoxic  factors 
are  at  work,  and  lead,  in  extreme  cases,  to  what  resembles  a  congestive 

^  Amer.  Jour.  Med.,  Sc,  1911,  xxiii,  509. 
2  Gaz.  d.  Hop.,  vol.  xiii,  p.  315. 
VOL.  IV — 22 


338  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

type  of  scleroderma  (Bulil).  This  is  well  shown  in  the  cases  reported  by 
Ogle/  and  by  Groedel  IP  of  a  huge  aneurism  of  the  subclavian  artery, 
which,  by  pressing  upon  and  obliterating  the  axillary  artery  and  vein 
and  brachial  plexus  (Ogle's  case),  led  to  an  enormous  tumefaction  with 
intense  cyanosis  and  clubbing,  strictly  confined  to  the  arm  and  fingers 
of  the  affected  side. 

Clubbing  of  pulmonary  or  hepatic  origin,  and  that  of  chronic  osteo- 
arthropathy (with  proliferative  changes  in  the  shafts  of  the  long  bones), 
has  been  sharply  distinguished  from  that  of  congenital  cardiac  disease 
on  the  ground  that  there  is  in  the  latter  cases  no  new  formation  of  bone 
(Ebstein^)  and  that  the  cause  of  the  clubbing  of  pulmonary  cases 
is  absorption  of  toxins  from  the  lung.  Increased  bone  formation  in  the 
clubbed  finger  ends  of  the  cardiac  cases  has  been  recorded,  however,  by 
Bamberger,  Janeway,^  Groedel  II,  and  Miller.^  Further,  a  case 
has  been  reported  by  Batty  Shaw  and  Cooper^  in  which  in  the 
entire  absence  of  pulmonary  disease,  signs  of  septal  defect,  with  deep 
cyanosis,  polycythemia  and  clubbing  were  combined  with  thickening 
of  the  shafts  of  the  tibia  and  other  long  bones  as  well  as  of  the  clubbed 
terminal  phalanges  (a;-ray  examination).  This  indicates  that  in  this  case 
at  least  of  congenital  cyanosis  we  are  dealing  with  a  milder  grade  of 
one  of  the  essential  symptoms  of  Marie's  disease. 

The  so-called  hippocratic  finger  ends  are  broadened  laterally  and 
are  slightly  flattened,  of  bulbous  appeai^ance,  with  distally  curved  nails, 
(compare  the  more  elongated  finger  ends  and  side  to  side  convexity  of 
the  marked  pulmonary  cases) .  The  nails  are  often  shortened  and  without 
lunulse  and  may  be  irregularly  thickened  from  areas  of  vascularization 
or  thrombotic  processes  in  the  matrix  below.  Microscopically,  increase 
in  the  soft  tissue  and  often  in  the  fat  (deficient  aeration)  is  conspicuous; 
surprisingly  little  tissue  change  is  sometimes  manifest. 

That  direct  admixture  of  venous  blood  is  more  important  in  the 
production  of  clubbing  than  pulmonary  obstruction  alone,  is  rather 
strikingly  suggested  by  the  fact  that  in  our  series,  among  40  cases  of 
pulmonary  stenosis  with  open  ventricular  septum  and  closed  foramen 
ovale,  clubbing  occurred  in  20,  while  in  11  cases  with  closed  ventricular 
septum  and  open  foramen  it  occurred  in  3;  and  in  7  cases  of  pulmonary 
stenosis  with  septum  and  foramen  both  closed,  it  was  not  present  once. 

Cyanosis  RetincB  sive  Ocidi. — Cyanosis  of  the  retina  was  described 
and  figured  in  Liebreich's  Atlas,  in  1863.  Eighteen  published  cases  were 
collected  by  Posey ^  in  1905,  and  Holloway^  has  brought  the  number 
recorded  to  27.  Ocular  changes  would  probably  be  found  in  nearly  all 
cases  of  cyanosis  if  the  eye  grounds  were  examined,  and  this  should 
always  be  done  for  diagnostic  reasons;  Babinski  reported  a  case  in  which 

1  Tr.  Path.  Soc,  London,  1859,  x,  103. 

2  Munchen.  med.  Wchnschr.,  1906,  liii,  264. 

3  Deutsch.  Arch.  f.  klin.  Med.,  1906,  p.  66. 
*  Amer.  Jour.  Med.  Set.,  1903,  cxxvi,  563. 

^  Trans.  Amer.  Fed.  Soc,  1904,  xvi,  p.  267. 
6  Clin.  Soc.  Trans.,  1907,  vol.  xl,  p.  260. 
^  Amer.  Jour.  Med.  Sci.,  1905,  cxxx,  415. 
8  New  York  Med.  Jour.,  1912,  xiv,  p.  71. 


CONGENITAL  CARDIAC  DISEASE  339 

dilatation  of  retinal  vessels  preceded  the  appearance  of  cyanosis;  so 
also  Carpenter. 

In  this  condition  ophthalmoscopic  examination  reveals  marked  changes 
in  the  optic  disk,  congestive  and  secondarily  inflammatory  in  character. 
The  disk,  itself,  is  unduly  reddened  or  bluish,  or  sometimes  hazy  and 
swollen  (neuroretinitis),  aiid  is  traversed  by  numerous  previously 
invisible  capillaries,  and  by  greatly  broadened,  often  tortuous  veins 
which  sometimes  show  a  deep  reflex  stripe  along  their  surface,  and  are 
jBlled  with  dark  brownish  or  even  blackish-looking  blood.  The  arteries 
may  share  in  the  dilatation  and  violet  discoloration  (as  in  12  out  of  the 
27  cases  recorded),  or  they  may  be  quite  unchanged,  showing  up  in  sharp 
contrast;  or  again,  as  in  the  cases  of  Baquis  and  Stanglomeier,  they  may 
be  contracted.  Retinal  hemorrhages  are  common.  The  difference  in  the  \ 
appearance  of  arteries  and  veins  was  suggested  by  Nagel  as  diagnostic  I 
between  anomalies  due  to  admixture  of  currents  (septal  defects,  etc.), 
and  those  due  to  pulmonary  obstruction  only,  the  arteries  in  the  latter 
case  remaining  unaltered.  This  point  has  yet  to  be  substantiated  by 
postmortem  evidence. 

In  advanced  cases  the  ocular  changes  are  not  confined  to  the  disk 
but  involve  the  whole  eye,  which  bulges  outward  (exophthalmos)  and 
shows  marked  conjunctival  congestion  and  cyanosis.  Hemorrhages 
may  occur  into  the  vitreous  or  more  superficially,  and  rupture  of  the 
cornea  (Goldzieher^),  or  glaucoma,  from  congestion  of  the  ciliary  body, 
may  occur.  Severe  iridocyclitis  may  develop,  as  in  the  cases  of  Gold- 
zieher  and  Baquis.^  In  both  of  these  the  iris  turned  while  under  observa- 
tion from  a  bright  blue  color  to  a  yellowish  brown,  a  change  found  by  the 
ocular  microscope  to  be  due  to  extreme  congestion.  This  symptom  was 
the  more  remarkable  because  it  was  seen  to  disappear  after  death.  Baquis' 
case  was  a  boy  aged  eleven,  with  extreme  cyanosis  and  clubbing,  a 
blood  count  of  8,500,000  red  cells  and  pulmonary  stenosis  with  septal 
defect.  He  gives  a  careful  description  of  the  microscopic  findings  in  the 
diseased  eyeballs  and  adds  a  study  of  eight  other  cases  in  the  German 
literature. 

Dyspncea. — Investigation  into  the  respiration  of  cyanotic  cases  has 
been  carried  out  by  special  methods  of  precision  by  Rubow,^  and  Bie  and 
Maar.'*  They  show  that  neither  total  nor  vital  capacity  is  increased, 
probably  because  of  the  hypertrophied  heart  and  dilated  veins  which 
commonly  occupy  so  large  a  part  of  the  thorax,  but  that  the  essential 
compensatory  change  consists  of  an  increased  pulmonary  ventilation,  b}^ 
acceleration  of  the  respiratory  rate.  Dyspnoea  is  the  response  made  by 
the  centre  i.n  the  medulla-toirrita.tionJ3y:,.the.unaerated  biopd.  Peabody^ 
regards  it  as  due  to  increased  acidosis  with  increased  excitability  of  the 
respiratory  centre.  It  is  a  prompt  and  early  feature.  It  usually  sets  in 
before  the .  cyanotic^  color  is  manifest.  It  frequently  culminates  in  the 
so-called  dyspnceic  attacks,  which  in  their  typical  form  and  full  develop- 

1  Centralb.  f.  prak.  Heilkunde,  September,  1904. 

2  von  Graef's  Arch.  f.  Ophth.,  1908,  Ixi,  68. 

3  Deut.  Arch.  f.  klin.  Med.,  1908,  xcii,  255. 

^  Ibid.,  1910,  xcix,  382.  =  Arch.  Int.  Med.,  1914,  xiv,  236. 


340  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

ment,  are  seizures  of  extreme  respiratory  distress  usually  attended  by 
marked  cyanosis,  and  sometimes  by  unconsciousness,  in  which  death 
may  take  place.  Such  attacks  are  characteristic  of  all  severe  cases  of 
cyanosis  but  are  frequent  also  in  such  conditions  as  patent  ductus 
arteriosus  in  which  there  may  be  no  trace  of  cyanosis  except  at  these 
times.  Transient  cyanosis  of  this  type  has  been  described  by  Sebilleau^ 
as  La  Cyanose  Paroxystique  Congenitale.  Respiratory  failure  may  be 
evidenced  also  by  repeated  syncopes  or  by  anginal  crises. 

Polycythemia. — This  is  now  well  recognized  to  be  a  common  char- 
acteristic of  cyanotic  blood.  The  red  cells  frequently  number  7,500,000 
to  8,500,000,  per  cmm.  the  percentage  of  hemoglobin  is  raised,  and  in 
some  cases  the  red  cells  are  also  increased  in  size.  Counts  of  12,750,000 
are  reported  by  Murray  Leslie,^  10,000,000  by  Pick  and  Parkes  Weber, 
and  9,000,000  by  Vaquez  and  Quiserne.^  Bernstein^  reports  a  case  of 
congenital  cyanosis  coming  on  at  the  sixteenth  month,  with  death  at 
two  and  one-half  years,  in  which  the  red  cells  numbered  10,000,000;  the 
tricuspid  orifice  was  absent  and  the  right  heart  aplastic  Math  defective 
interauricular  and  interventricular  septa. 

The  increase  of  the  red  cells  is  generally  thought  to  be  of  the  nature 
of  a  compensatory  process,  and  has  been  compared  to  the  polycythemia 
of  high  altitudes.  Weil  describes  in  detail  a  microscopic  finding  in  two 
cyanotic  children  with  pulmonary  stenosis  in  whom  the  red  cells  numbered 
respectively  7,502,000  and  8,540,000.  The  hematopoietic  organs  and 
also  the  other  tissues  examined  were  crowded  with  vasoformative  cells 
and  embryonic  capillaries  and  the  bone  marrow  show^ed  a^  typical  ery- 
throblastic reaction.  Similar  appearances  have  been  noted  by  other 
observers  and  prove  that  this  feature  is  part  of  the  compensatory  mechan- 
ism of  the  organism  for  the  better  oxygenation  of  the  tissues. 

The  blood  count  differs  somewhat  at  different  parts  of  the  body  and 
according  to  w4iether  the  specimen  has  been  drawn  from  arteries,  veins, 
or  capillaries  but  it  remains  far  above  the  normal,  the  increase  in  red  cells 
remaining  an  absolute  and  not  a  relative  quantity  (Bie  and  Maar). 
That  is  to  say  in  these  cases  the  total  quantity  of  blood  in  the  vessels 
is  increased,  a  moderate  degree  of  hydremia  existing. 

Polycythemia  is  not  a  constant  feature  in  congenital  cyanosis,  it  is 
characteristic  rather  of  the  later  stages  of  the  disease,  and  while  favorable 
in  so  far  as  it  represents  an  attempt  at  compensation,  its  appearance 
points  to  a  grave  prognosis. 

Diagnosis. — The  cyanosis  of  congenital  cardiac  disease  must  be  differ- 
entiated from  a  number  of  other  forms.  Of  first  importance  among  these 
is  the  so-called  enterogenous  cyanosis^  (first  described -by  Stockvis  and 
other  Dutch  observers),  in  which  the  dark  color  of  the  blood  is  due  to  a 

1  These  de  Paris,  1895.  ^  p^oc.  Royal  Soc,  Clinical  Section,  1908,  i,  34. 

'  Corn-pie  rendu  de  la  Soc.  de  hiol.,  July  10,  1904. 

*  Ibid.,  1902,  p.  915. 

5  A  digest  of  enterogenous  cyanosis,  with  a  tabulated  statement  of  the  recorded 
cases  and  full  bibliography,  is  given  by  West  and  Clarke  (Lancet,  February  2,  1907). 
Other  important  articles  are  by  Hymans  van  der  Bcrgh  (Deulsch.  Archiv  f.  klin. 
Med.,  1905),  Oliver  {Lancet,  December  29,  1906),  Gibson  {Lancet,  July  14,  1906),  and 
Blackader  {New  York  Med.  Jour.,  March  16,  1907,  Ixxxv). 


CONGENITAL  CARDIAC  DISEASE  341 

sulph-hemoglobinemia  or  methemoglobinemia,  produced,  it  is  thought, 
by  the  action  of  hydrogen  sulphide  or  other  toxic  agents  upon  the  blood. 
Again,  certain  aniline  poisons  lead  to  a  methemoglobinemia  giving  a 
dark  discoloration  to  the  skin,  and  polycythemia  with  splenomegaly  is 
sometimes,  although  not  always,  associated  with  cyanosis.  These  con- 
ditions differ  from  congenital  cyanosis  in  the  slightly  different  tinge  of 
the  skin,  which  in  methemoglobinemia  is  of  a  gra^dsh  hue,  in  poly- 
cythemia with  splenomegaly  of  a  more  florid  aspect,  by  the  absence  of 
cardiac  signs,  by  the  presence  of  intestinal  symptoms,  and  by  the  history 
of  a  toxic  factor  or  the  presence  of  enlargement  in  spleen  and  liver.  In 
methemoglobinemia  the  red  cells  are  not  increased  in  number.  Other 
conflitions  leading  to  cyanosis  with  clubbing  are  pulmonary  emphysema, 
bronchiectasis,  and  adherent  pericardium. 

CLASSIFICATION. 

Many  attempts  have  been  made  to  reduce  cardiac  anomalies  to  a 
scientific  classification,  but  none  have  been  entirely  successful.  The 
most  logical  arrangement  is  undoubtedly  one  based  upon  the  stage  of 
development  at  which  the  defect  has  occurred,  differentiating  those 
cases  due  to  arrest  of  growth  from  those  originating  at  a  period  of  intra- 
uterine life  and  apparently  caused  by  fetal  disease.  Our  knowledge  of 
the  development  of  the  heart  is  still  too  limited  to  permit  of  a  complete 
classification  on  this  basis,  and,  on  the  other  hand,  the  etiology  of  a 
given  condition  is  often  impossible  to  decide  in  the  individual  case.  More- 
over, a  grouping  based  on  development  alone  is  sometimes  unpractical, 
for  widely  different  pathological  results  may  ensue  from  arrest  of  different 
parts  of  the  heart  at  about  the  same  period  of  fetal  life.  For  these  reasons 
no  one  classification  will  be  found  adequate  in  its  practical  application, 
and  it  would  almost  seem  that  a  grouping  "on  mixed  principles"  is  the 
only  one  under  which  all  the  cases  can  be  satisfactorily  placed. 

The  631  cardiac  defects  here  studied  present,  either  as  the  primary 
lesion  or  as  a  complicating  condition,  illustrations  of  practically  all  the 
cardiac  anomalies  known.  In  the  tables^  of  relative  frequency,  age,  sex, 
etc.,  pages  342  and  343,  an  attempt  is  made  to  arrange  these  cases  in  as 
logical  an  order  as  possible.  It  represents  an  attempt  at  a  detailed  classi- 
fication based  on  strictly  anatomical  principles  and  on  development  so 
far  as  this  is  known,  but  without  regard  to  the  etiology,  which  is  so  often 
obscure.  Thus  the  group  of  stenosis  and  atresias  of  valvular  orifices  is 
subdivided,  not  into  inflammatory  and  developmental  forms  and  their 
subdivisions,  with  or  without  transposition  or  rechtslage  (Rauchfuss, 
Vierordt),  but  on  anatomical  considerations  only,  with  regard  to  the 
seat  of  the  stenosis  and  the  presence  or  absence  of  septal  defects. 

1  Note. — "Double"  indicates  a  systolic  with  a  diastolic  murmur.  The  sex  is  not 
always  mentioned  in  the  records  of  cases.  The  nmnbers  in  these  columns  do  not, 
therefore,  always  correspond  to  the  totals  in  the  chart.  The  data  given  as  to  age, 
sex,  postmortem  findings,  and  clinical  aspects  refer  to  the  cases  in  the  first  column  in 
the  chart.  This  column  is  repeated  in  the  third  to  last  column,  and  this,  added  to 
the  second  to  last  column,  gives  the  total  relative  frequency  of  each  defect,  whether 
classified  as  primary  lesion  or  as  complicating  other  defects. 


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344  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

The  majority  of  cardiac  defects  are  complicated,  and  it  is  often  difficult, 
sometimes  impossible,  to  say  which  is  the  primary  lesion;  many  of  the 
eases  present  other  anomalies  of  equal  importance  with  those  with  which 
they  are  grouped.  A  cross-index  has  therefore  been  made  in  the  two 
columns  next  to  the  end,  and  the  total  relative  frequency  is  to  be  found 
in  the  last  column  of  the  chart. 

ANOMALIES  OF  THE  PERICARDIUM. 

Absence  or  Defect. — The  Pericardium  may  be  entirely  absent,  as  in 
some  forms  of  ectopia  cordis,  or  its  parietal  layer  may  be  more  or  less 
defective.  Some  forty-two  cases  are  on  record  and  the  subject  has  recently 
been  reviewed  by  Ebstein,^  Plaut,^  McGarry,^  and  Cameron.  The  defect 
always  involves  the  left  side  of  the  sac  but  it  may  vary  from  a  localized 
hole  with  smooth  edges  lying  over  the  pulmonary  artery  opposite  the 
root  of  the  left  lung  and  communicating  with  the  left  pleura,  to  complete 
absence  of  the  parietal  layer,  the  heart  lying  in  the  anterior  mediastinum, 
without  any  serous  envelope.  In  all  the  cases  recorded  except  the  four 
above  mentioned,  the  whole  left  and  anterior  walls  of  the  pericardium 
are  wanting,  and  the  heart  and  left  lung  lie  in  a  common  cavity,  the  left 
pleura  being  continuous  with  the  epicardium  and  the  anterior  and  right 
walls  of  the  sac  being  represented  by  one  or  more  rudimentary  folds; 
in  the  best  developed  cases  two  folds,  one  sagittal  and  one  crescentic; 
spring  from  the  diaphragm  and  are  inserted,  the  one  into  the  anterior 
mediastinum  and  the  other  posteriorly  at  or  near  the  root  of  the  left 
lung;  in  the  more  extreme  cases  the  diaphragm  is  free  and  a  serous  fold 
encircles  the  base  of  the  heart  with  the  great  trunks;  in  complete  defects 
no  trace  of  the  parietal  layer  may  exist,  or  it  may  be  represented  by  a 
few  fatty  appendices  arranged  fringe-like  about  the  base  of  the  heart. 
The  left  phrenic  nerve  maintains  its  relationship  to  the  pleuropericardial 
septum,  running  down  parallel  to  the  border  of  the  sagittal  rudimentary 
fold  w^hen  this  is  present,  and  is  displaced  to  the  right  in  a  degree  increas- 
ing with  the  degree  of  the  defect.  This  anomaly  in  the  course  of  the 
nerve,  and  the  serous  investment  of  the  margins  of  the  defect,  are  points 
diagnostic  of  its  congenital  origin. 

Pathogenesis. — An  explanation  of  the  condition  is  to  be  sought  toward 
the  end  of  the  fifth  week  of  fetal  life,  when  the  pericardial  begins  to  be 
separated  from  the  pleural  coelom.  At  this  time  the  so-called  pulmonary 
ridge,  an  elevation  from  the  walls  of  the  ducts  of  Cuvier,  encircles,  and 
gradually  obliterates,  the  canal  of  communication  between  the  two 
cavities,  and  descends  to  the  septum  transversum  or  primitive  diaphragm 
as  the  pleuropericardial  septum.  Under  normal  conditions  the  left  duct 
of  Cuvier  atrophies  in  early  fetal  life.  Pericardial  defect,  with  its  in- 
variable relation  to  the  left  pleural  cavity,  may  well  be  due,  as  suggested 
by  Perna,  to  a  too  early  atrophy  of  this  structure,  dependent  upon  some 
anomaly  of  circulation  in  the  great  venous  trunks,  the  arrested  develop- 

1  Munchen.  med.  Wchnschr.,  1910,  Ivii,  522. 

2  Frankf.  Zeitsch.f.  Path.,  1913,  xii,  141. 

3  Anat.  Record,  1914,  February  20. 

^  Trans.  Chicago  Path.  Soc,  1914,  ix,  p.  148. 


CONGENITAL  CARDIAC  DISEASE  345 

ment  of  the  pulmonary  ridge  resulting  leading  to  a  localized  defect  (pleuro- 
pericardial  foramen),  or  to  the  more  or  less  complete  absence  of  the 
pleuropericardial  septum.  In  one  of  Keith's  cases,  a  defect  of  the  left 
pleuroperitoneal  membrane  is  also  to  be  concluded,  for  the  heart,  left 
lung,  liver,  spleen,  and  stomach  lay  in  the  left  pleuropericardial  cavity. 

Clinical  Aspects. — The  defect  in  itself  has  no  direct  effect  upon  the 
heart,  the  patients  reaching  adult  life,  and  sometimes  even  attaining 
advanced  age  without  any  untoward  sign.  Its  clinical  significance 
depends  chiefly  upon  two  factors:  First  of  these  is  the  abnormal  juxta- 
position of  the  heart  and  left  lung,  whereby  the  heart  is  exposed,  without 
the  protection  of  its  serous  envelope,  to  the  many  inflammatory  changes 
prone  to  attack  the  pleura;  some  cause  also,  whether  traction  from 
adhesions  with  the  constantly  pulsating  heart  or  friction  with  the  surface 
of  this,  appears  to  act  deleteriously  upon  the  left  lung  and  pleura,  pre- 
disposing these  to  disease.  Among  the  forty  cases  recorded  the  incidence 
of  left-sided  pulmonary  complications  is  extremely  large.  Picchi's 
two  patients  died  of  left  lobar  pneumonia,  Weisbach's  of  left  purulent 
pleurisy,  Powell's  of  left  pneumothorax.  Plant's  second  case  and  Faber's 
of  acute  left-sided  pleurisy  and  Baly's,  Hughes',  and  Saxer's  of  pulmonary 
tuberculosis. 

Secondly,  the  cor  mobile  resulting  frorn  the  lack  of  pericardial  attach- 
ment to  the  diaphragm  and  mediastinal  structures,  may  lead  to  sudden 
kinking  of  the  great  vessels  resulting  not  only  in  s3^mptoms  of  precordial 
distress  (Ebstein),  but  apparently,  in  one  instance  at  least  in  death 
itself.  In  the  case  recorded  by  Boxall  death  occurred  on  the  third  day 
after  confinement,  apparently  from  the  sudden  slipping  of  the  heart's 
apex  out  of  a  low-walled  pouch  formed  by  two  rudimentary  folds  attached 
to  the  diaphragm,  in  the  altered  intrathoracic  pressure  following  delivery. 
Thirty  hours  before  death  urgent  dyspnoea,  collapse,  and  a  systolic 
murmur  over  the  precordium  suddenly  appeared.  Pulmonary  thrombosis 
was  suspected,  but  was  not  confirmed  at  autopsy,  when  the  heart  was 
found  in  the  left  pleura.    The  number  of  fatalities  is  high. 

Diagnosis. — This  is  difficult,  but  does  not  seem  impossible.  The 
greatly  increased  mobility  of  the  heart,  its  occasional  hi^'pertrophy  either 
from  this  cause  or  from  the  traction  of  inflammatory^  fibrous  tissue  bands 
attaching  it  to  the  left  pleura  and  diaphragm,  and  its  frequent  displace- 
ment to  the  left  through  such  traction  or  from  similar  causes  are  the 
chief  points.  In  spite  of  its  rarity,  pericardial  defect  should  always  be 
considered  in  the  light  of  the  .T-ray  findings. 

In  Faber's  case,  a  man  of  fifty-one  years,  in  good  health  until  five  weeks 
before  death,  when  an  extensive  left  pleural  eft'usion  developed,  the  heart 
was  only  moderately  displaced  to  the  right  before  aspiration,  although 
the  left  chest  was  entirely  dull;  after  the  withdrawal  of  2200  cc.  of 
fluid  the  apex  was  evident  in  the  third  and  fourth  interspaces  in  the 
left  anterior  axillary  line,  and  at  a  later  aspiration  of  3100  cc,  the  heart 
was  felt  in  this  region  by  the  aspirating  trocar.  These  facts  supplied 
a  basis  for  a  correct  diagnosis. 

Unattached  Pericardium. — Turner  describes  an  adult  male  subject 
in  whom  the  parietal  pericardium  was  perfectly  free  from  continuity 


346  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

with  the  central  tendon  of  the  diaphragm  or  other  structure,  and  was 
attached  only  about  the  base  of  the  great  vessels,  closely  embracing  the 
heart,  which  could  be  drawn  completely  out  of  the  chest  (cor  viohile),  . 
the  inferior  vena  cava  lay  free  in  the  thorax  for  an  inch  before  piercing 
the  pericardium.  A  similar  condition  was  observed  in  the  w^alrus  and 
was  thought  here  to  be  normal. 

Diverticuluin  or  Hernia  Pericardii. — This  consists  in  a  localized  bulging 
of  the  serous,  or  serous  and  fibrous,  coats  of  the  parietal  layer,  whereby 
a  thin-walled  cyst  is  formed,  communicating  with  the  pericardial  cavity 
either  directly  by  a  narrow  orifice,  or  by  a  tubular  canal.  An  interesting 
example  was  seen  by  the  writer  in  the  Museum  at  Bologna,  from  a  case 
published  by  Coen.^  In  this  specimen  a  unilocular  chamber  of  a  capacity 
of  40  cc,  formed  by  the  protrusion  of  the  serosa,  projected  from  the 
right  border  of  the  parietal  pericardium,  as  a  kidney  shaped  tumor 
17.5  cm.  in  circumference,  and  communicated  with  the  interior  of  the 
pericardial  sac  by  a  minute  orifice  lying  at  the  bottom  of  a  small  fossa. 
Coen  gives  an  analysis  of  ten  other  cases  in  the  literature. 

This  condition  is  of  little  clinical  importance,  usually  remaining 
latent.  Secondary  pathological  changes,  such  as  calcification  or  fibrosis 
of  the  cyst  walls,  or  occlusion  of  its  orifice  and  consequent  distension  of 
its  cavity  may  supervene,  and  make  it  an  impediment  to  the  heart's 
action.  The  possibility  of  a  pericardial  diverticulum  should  not  be  over- 
looked in  the  diagnosis  of  mediastinal  tumors. 

DISPLACEMENTS  OF  THE  HEART. 

Ectopia  Cordis. — By  this  term  is  understood  a  displacement  so  that 
the  heart  passes  out  of  the  thorax,  and  comes  to  lie  either  upon  the  outer 
surface  of  the  body  or  in  the  abdominal  cavity.  Full  bibliographical 
studies,  with  reports  of  cases,  are  given  by  Jones,^  and  by  Ellis.^  The 
latter,  following  Rauchfuss,  recognizes  three  forms,  (1)  cervical  heart, 
in  which  the  organ  lies  high  up  in  the  neck;  (2)  pectoral  heart  with 
fissure  of  the  sternmn,  and  (3)  abdominal  heart  in  which  the  heart  is 
projected  through  a  defect  in  the  diaphragm  into  the  abdominal  cavity. 

The  existence  of  cervical  heart,  or  of  pectoral  heart  with  fissure  of 
the  upper  part  of  the  sternum  and  absence  of  pericardium,  is  not  com- 
patible with  life;  in  abdominal  heart  on  the  other  hand  life  is  not  neces- 
sarily shortened  (witness  Deschamps'  case  of  a  healthy  soldier  whose 
heart  occupied  the  position  of  the  left  kidney  in  the  lumbar  region), 
and  in  pectoral  heart  with  inferior  sternal  fissure  and  pericardium  present, 
it  might  with  due  care  be  indefinitely  prolonged.  In  Goode's^  patient, 
with  fissure  of  the  sternum  below  the  manubrium,  the  heart  was  oiled 
every  three  hours  and  kept  covered  by  a  cardboard  box,  and  the  child 
did  very  well  for  a  time.  Ellis  quotes  a  case  in  which  a  thin  membrane 
covering  the  heart  sloughed  on  the  fifteenth  day,  and  the  outer  skin 

^  Bull,  della  Soc.  Med.  Chi.  di  Bologna,  1885,  xv,  Fasc.  1. 

2  Trans.  Path.  Soc,  London,  1869,  xx. 

3  Proc.  Path.  Soc,  Philadelphia,  1906,  p.  36. 
*  Virginia  Med.  Semi-Monthly,  1904,  p.  555. 


CONGENITAL  CARDIAC  DISEASE  347 

covering  the  organ  was  successfully  united  by  sutures.  He  suggests  that 
such  operative  interference  might  be  tried  in  suitable  cases  where  the 
visceral  pericardium  is  present. 

Dextrocardia. — This  term  is  applied  clinically  to  all  cases  in  which  the 
heart  is  found  displacM  tQ.the..nght.side.oLthe_thm3-x.  Pathologically 
two  conditions  must  be  distinguished .  In  the  one  the  heart  is  simply 
displaced  to  the  right  as  a  result,  usually,  of  acquired  disease  {De.rtro- 
versio  cordis).  In  the  other  the  organ  is  also  more  or  less  altered  in  its 
position  in  the  thorax,  so  that  the  apex  comes  to  point  to  the  right.  In 
these  latter  cases  two  forms  must  again  be  recognized : 

A.  The  heart  is  not  transposed,  but  appears  to  have  undergone  a 
simple  rotation  from  left  to  right  on  its  vertical  axis,  so  that  its  left 
chambers  come'  to  lie  more'  anteriorly  and  its  right  chambers  more  pos- 
teriorly. The  apex  points  to  the  right  but  is  formed  not  of  the  left  but 
of  the  right  (venous)  ventricle,  which  remains  on  the  right  side  and 
receives  blood  from  the  right  (venous)  auricle  into  which  the  venae  cavse 
empty.  This,  which  is  not  a  true  transposition,  is  the  condition  present 
in  the  majority  of  the  cases  of  congenital  dextrocardia.  It  is  well  illus- 
trated by  Grunmach's^  case  (See  Fig.  21.) 

B.  The  heart  may  he  completely  reversed  upon  itself,  those  parts 
normally  upon  the  left  coming  to  lie  on  the  right  side,  but  the  relation 
of  the  various  structures  to  each  other  remaining  unchanged,  so  that  a 
complete  mirror  picture  of  the.  normal,  heart  results.  Here  the  apex, 
pointing  to  the 'bright,  is  formed  of  what  was  normally  the  left  ventricle, 
which  now  lies  on  the  right  side  and  communicates  with  the  right 
(normally  left)  auricle  which  receives  the  pulmonary  veins  and  is  struc- 
turally the  systemic  auricle.  In  complete  situs  inversus  this  "mirror" 
condition  is  the  rule,  but  true  transposition  of  the  heart  only,  without 
situs  inversus  is  exceedingly  rare,  the  only  cases  we  know  of  on  record 
being  these  of  Graanboom^  and  Rokitansky. 

The  etiology  of  these  two  t^^pes  of  congenital  dextrocardia  differs 
widely.  NageP  reviews  six  cases  with  clinical  and  autopsy  reports  in 
the  literature,  and  demonstrates,  with  the  aid  of  diagrams,  that  five  of 
these,  namely,  those  by  Grunmach,  Geipel,  Lowenthal,  Barmwerth  and 
himself,  belong  to  the  type  A  above,  in  which  the  heart  is  not  trans- 
posed, but  presents  simply  a  persistence  of  the  embryonic  stage,  in  which 
the  apex  was  formed  by  the  right  half  of  the  common  ventricle.  The 
fact  that  in  all  five  of  these  cases  the  grave  anomalies  of  pulmonary 
stenosis  and  ventricular  septal  defect  were  associated,  affords  convincing 
proof  that  a  primary  arrest  of  development  had  occurred. 

In  the  sixth  case,  on  the  other  hand,  that  by  Graanboom,  in  which 
there  was  true  transposition  of  the  heart,  and  the  septiun  was  entire,  the 
probable  explanation  is  to  be  sought,  not  in  an  arrest  of  development, 
but,  with  the  causes  of  transposition  in  general,  in  the  altered  relation 
of  the  embryo  to  the  primitive  chorionic  villi. 

Congenital   dextrocardia,   unassociated  with   other   anomaly,   is   not 

^  Berlin,  klin.  Wchnschr.,  1890,  No.  2. 

2  Zeitschr.  f.  klin.  Med.,  1891,  Bd.  18,  2. 

^  Deut.  Arch.  f.  klin.  Med.,  vol.  xcvi,  p.  552. 


Fia.  21 


1.  Normal  heart.  .-1,  caval  auricle;  B,  pul- 
monary veins  auricle;  C,  right  ventricle  (caval 
blood  ventricle) ;  D,  left  ventricle  (pulmonary 
blood  ventricle). 


2.  Embryonic  heart.  A,  caval  auricle;  B,  pul- 
monary veins  auricle;  C,  right  ventricle  (caval 
blood  ventricle) ;  D,  left  ventricle  (pulmonary 
blood  ventricle). 


3.  TNIirror  picture  of  normal  heart.  A,   caval 

auricle;     B,   pulmonarj^   veins   auricle;  C.   right 

ventricle    (caval   blood   ventricle) ;     D,  left   ven- 
tricle (pulmonary  blood  ventricle). 


4.  Pure  congenital  dextrocardia.  A,  caval 
auricle;  B,  pulmonarj'  veins  auricle;  C,  caval 
blood  ventricle;  D,  pulmonary  blood  ventricle. 
Note  similarity  to  No.  2. 


5.  Graanboom's  case  of  congenital  dextrocardia 
(mirror  picture).  A,  caval  auricle;  B,  pulmon- 
ary blood  auricle;  C,  caval  blood  ventricle;  D, 
pulmonary  blood  ventricle. 


6.  Grunmach's  case  of  pure  congenital  dextro- 
cardia (arrest  in  embryonic  stage).  E,  vena  cava 
superior;  F,  ventriculum  dextrum;  G,  aorta;  H , 
ventriculum  sinistrum. 


1,  2,  3,  4.  Diagrams  (1)  shonang  normal  heart;  (2)  embryonic  heart;  (3)  mirror  picture  of  normal 
heart;  (4)  heart  in  usual  type  of  pure  congenital  dextrocardia;  (5)  from  Graanboom's  case  of  pure 
dextrocardia  (mirror  picture).  Note  that  the  apex  is  formed  by  the  left  (pulmonary  blood)  ventricle. 
(From  Nagel's  article  on  Pure  Congenital  Dextrocardia  in  the  Deutsches  Archiv.  f.  klin.  Med.,  xcvi,  p. 
572.)  (6)  Grunmach's  case  of  pure  congenital  dextrocardia;  arrest  of  development,  persistent  embrj-oni, 
stage.  Note  that  the  apex  is  formed  by  the  right  (caval  blood)  ventricle.  (From  his  article  in  the  Ben. 
klin.  Wchnschr.,  1890,  page  22.)  The  auricles  are  designated  "caval"  and  "pulmonary"  (according  to  tae 
veins  they  receive) ,  instead  of  right  and  left,  in  order  to  show  the  difference  in  situation  in  the  mirror  picture 
and  in  Graanboom's  case,  from  that  of  the  usual  type  of  pure  congenital  dextrocardia  (Grunmach's  jase). 


CONGENITAL  CARDIAC  DISEASE 


349 


itself  of  clinical  significance  and  may  be  discovered  accidentally  with  the 
heterotaxy  that  usually  accompanies  it,  in  a  perfectly  healthy  subject, 
by  physical  and  .r-ray  examination,  which  reveals  the  normal  area  of 
cardiac  dulness  on  the  right  side,  with  corresponding  location  of  sounds 
and  fluoroscopic  findings.  Transposition  of  the  heart,  of  the  type  usually 
associated  with  situs  inversus  viscerum  (Graanboom's  case)  yields  a 
characteristic  electrocardiogram  (Neuhof,^  Lewis^),  in  that  Lead  I,  shows 
a  reversal  of  all  curves,  and  Leads  II  and  III,  replace  each  other.  This 
supplies  a  diagnostic  point  between  the  transposed  and  non-transposed 
forms  of  Congenital  Dextrocardia  and  also  distinguishes  the  former  from 
a  dextroversio,  in  which  the  electrocardiogram,  although  atypical,  is  not 
reversed.  The  curves  in  this  latter  condition  have  been  studied  by 
Neumann.^ 


Fig.  22 


1.  Electrocardiogram  from  a  case  of  congenital  dextrocardia  with  transposed  (mirror-picture)  heart. 
Note  that:  Lead  /,  is  completely  reversed  in  that  P,  R,  and  T,  normally  directed  upward  in  this  lead, 
here  point  downward.    Lead  //,  has  changed  places  with  Lead  III. 

2.  Electrocardiogram  from  a  case  of  dextro-versio-cordis,  i.  e..  simple  displacement  of  the  heart  to 
the  right.  Here  there  is  no  reversal  of  leads,  but  Lead  I  especially  deviates  from  the  normal  in  Q 
and  R.      (From  Clinical  Electrocardiography,  by  Thomas  Lewis,  I-ondon,   1913.) 

Incomplete  Heterotaxy. — Of  interest  are  three  cases  reported  by 
Hickman,  Royer  and  Wilson,^  and  McCrae,  of  transposition  of  the 
viscera  with  only  partial  transposition  of  the  heart,  which  maintained 
its  normal  position  with  apex  pointing  to  the  left,  the  ventricles  and  auriculo- 
ventricular  ostia  unchanged,  but  the  auricles  and  great  arterial  trunks 
transposed,    and   the   auricular   septum   defective.      In  McCrae's   and 

1  Jour.  Amer.  Med.  Assn.,  1913,  Ix,  p.  1064. 

2  Clinical  Electrocardiography,  191.3,  p.  100. 

^  Deutsch.  nied.  Wchnschr.,  1912,  xxxviii,  p,  1920, 
^  Arch.  Pediat.,  1908,  xxv,  882. 


350  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

Hickman's  cases  the  pulmonary  artery  was  atresic,  in  that  by  Royer  and 
Wilson  it  was  stenosed,  and  arose  from  the  right  ventricle  with  the 
aorta  but  in  transposed  relation.  The  ventricular  septum  was  entire 
in  McCrae's  case,  defective  in  the  other  two. 

Intrinsic  Displacement  of  Chambers. ^Four  interesting  examples, 
in  anencephaly  and  ectopia  cordis,  of  displacement  of  the  chambers  upon 
each  other,  as  a  result  of  extraneous  mechanical  force  exerted  on  the 
heart  after  it  was  fully  formed,  are  reported  by  Jane  Robertson.^ 

ANOMALIES  OF  THE  HEART  AS  A  WHOLE. 

Acardia,  Hemicardia. — Where  grave  interference  with  the  circulation 
occurs  at  a  very  early  embryonic  period,  the  heart  may  not  develop  at 
all  (acardia),  or  it  may  be  rudimentary  (hemicardia).  Such  a  condition 
usually  develops  in  one  member  of  a  uniovular  twin  pregnancy  in  which 
anastomosis  of  the  vessels  of  .the  two  individuals  can  take  place  through 
their  common  placenta. 

All  degrees  of  acardia  may  occur  and  from  the  developmental  stand- 
point the  findings  are  extremely  interesting.  Thus  Campbell  and  Shep- 
herd report  a  case  in  which  the  circulation  had  been  interfered  with 
before  the  heart  had  formed  at  all,  and  in  which  the  two  dorsal  arteries 
passing  from  the  umbilical  opening  toward  the  head  of  the  amorphous 
monster,  represented  a  persistence  of  the  early  vitelline  circulation. 
Kehrer^  adds  to  an  analysis  of  thirteen  cases  from  the  literature  an  account 
of  a  case  of  his  own  which  is  remarkable  for  the  advanced  state  of  the 
rudimentary  organ.  Cases  not  included  in  Kehrer's  review  are  reported 
by  Schubert^  and  by  Nacke  and  Benda.'' 

Multiple  Hearts. — This  phenomenon  is  commoner  in  animals.  An 
explanation  is  perhaps  to  be  sought  in  an  irregular  division  of  the 
omphalomesenteric  or  vitelline  veins,  from  which  the  first  heart  milage 
is  derived. 

Bifid  Apex. — This  may  occur  without  other  anomaly  or  complicating 
grave  defects.  The  apex  of  the  early  embryonic  heart  during  its  amphib- 
ian stage,  is  bifid;  a  condition  to  be  ascribed  to  the  rapid  downward 
growth  of  the  apices  of  both  ventricles  at  this  time,  so  that  a  deep  inter- 
ventricular groove  or  cleft  is  formed.  Obliteration  of  this  cleft  takes 
place  in  embryos  over  11  mm.  long,  by  the  development  downward  of 
the  embryonic  apex,  which  occurs  simultaneously  with  the  closure  of  the 
interventricular  foramen.  In  the  adult  mammalian  heart  bifid  apex  is 
thus  to  be  ascribed  to  a  persistence  of  the  interventricular  groove  (Gegen- 
baur),  due  to  an  arrest  of  development  of  the  embryonic  apex,  as  is 
evidenced  by  the  absence  of  the  muscular  vortex  normally  present  here, 
each  apex  in  the  bifid  state  being  formed  independently  from  musculature 
derived  respectively  from  the  musculospiral  and  sinospiral  bands  (Mall)  .^ 
A  deeply  bifid  apex  is  normal  in  the  dugong;  it  occurs  in  3  of  Theremin's 
106  cases  and  in  15  of  our  series. 

1  Jour.  Path,  and  Bad.,  1913,  xviii,  211.  ^  ^^^h.  J.  Gynak.,  1908,  Ixxxv,  121. 

3  Monatsch.  f.  Geburts.,  1908,  xxviii,  3.  ^  Zentralbl.  f.  Gynak.,  1907,  p.  17. 

5  Anat.  Rec,  1912,  vi,  167. 


CONGENITAL  CARDIAC  DISEASE  351 

Diverticulum. — ^The  heart  may  be  prolonged  into  a  hollow  process. 
Arnold"-  reports  a  female  child,  aged  two  and  a  half  months,  a  subject 
of  congenital  lues,  in  whom  the  apex  of  the  left  ventricle  ran  out  into  a 
hollow  process  which  bent  around  hke  a  hook,  its  blind  end  projecting 
upward  and  to  the  left.  In  Koller-Aeby's^  case  it  formed  a  pulsating 
process  with  muscular  walls  descending  from  the  apex  of  the  left  ventricle 
through  a  defect  in  the  diaphragm  to  form  part  of  an  umbilical  hernia. 
Diverticulum  of  the  heart  is  rare  and  of  no  clinical  significance. 

Primary  Congenital  Hypertrophy. — Recently  a  number  of  observations 
have  been  published  in  which  hypertrophy  of  the  heart  existed  at  birth. 
In  1898,  Simmonds^  reported  the  first  clear  case  of  primary  congenital 
hypertrophy.  In  a  newly  born  child  which  died  during  a  protracted 
labor  all  the  organs  including  the  kidney  were  normal,  but  the  heart  was 
greatly  enlarged.  It  weighed  44  gm.  (normal  weight  19  to  20  gm.); 
the  right  ventricle  was  0.75  to  1  cm.,  the  left  1  to  1.25  cm.  thick.  The 
papillary  muscules  were  small  and  took  no  part  in  the  hypertrophy, 
thus  indicating  that  this  was  not  caused  by  overwork.  Simmonds 
suggests  that  the  hypertrophy  may  have  been  due  either  to  some  cause 
in  early  embryonic  life,  which  may  later  pass  away,  or  to  Mrchow's 
diffuse  myomatous  infiltration.  Other  typical  cases  of  congenital 
hypertrophy  without  valvular  disease,  nephritis,  or  apparent  cause,  are 
reported  by  Effron,'*  Kalb,"^  and  Ratner.^ 

Congenital  Rhabdomyoma. — An  interesting  problem  is  presented  by 
the  occurrence  in  infants  or  young  children  of  embryonic  muscle  tumors 
of  the  heart  wall  associated,  in  the  majority  of  the  cases,  with  sclerosis 
of  the  cerebral  cortex.  The  condition  was  first  described  by  von  Reckling- 
hausen and  by  Virchow  and  was  first  reported  on  this  Continent  by 
Knox  and  Schorer.^  Wohlbach^  has  recently  studied  the  11  cases  on 
record,  and  has  added  an  observation  of  his  own  of  an  instance  in  which 
a  rhabdomyoma  of  the  wall  of  the  right  ventricle  was  associated  with 
multiple  nests  of  neuroglia  in  the  spinal  meninges.  The  child,  a 
female,  with  hydrocephalus  and  spina  bifida  and  paralysis  of  the  lower 
half  of  the  body  from  birth,  died  of  scorbutus  at  ten  months.  The 
heart  was  very  large,  weighing  72.5  gm.,  with  hypertrophied  papillary 
muscles.  Just  below  the  pulmonary  valve  an  ovoid  nodule,  1.7  x  4  cm. 
wide,  of  grayish-red  color  and  elastic  consistence,  lay  imbedded  in  the 
interventricular  septum  and  in  the  papillary  muscle  of  the  anterior 
tricuspid  valve  segment,  to  which  it  supplied  chordse.  Microscopically 
it  was  composed  of  a  delicate  reticulum  of  connective  tissue  supporting 
heterogeneous  cells  having  a  more  or  less  irregularly  striated  fibrillary 
matrix  and  containing  in  many  instances  large  intracellular  spaces  and 
peripherally  placed  nuclei,  the  whole  bearing  a  bizarre  resemblance  to  the 
modified  muscle  cells  (Purkinje  fibres)  of  the  adult  heart. 

An  explanation  of  this  remarkable  combination  of  congenital  cerebral 


1  Virchows  Arch.,  1894,  cxxxvii,  318.  ^  Arch.  f.  Gyndk.,  1907,  Ixxxii,  185. 

^  Munchen.  med.  Wchnschr.,  1898.  ^  Zurich  Thesis,  1903. 

s  Munich  Thesis,  1906.  ^  Berlin  Thesis,  1912. 

7  Arch.  Pediat.,  1906.  xxiii,  361.  ^  Jour.  Med.  Research.,  1907,  xvi,  495. 


352  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

sclerosis  with  rhabdomyoma  may  be  sought  in  the  fetal  malnutrition 
invariably  present,  which  may  have  led  to  the  vascular  degeneration 
that  must  underlie  such  changes  (Wohlbach). 

ANOMALOUS    SEPTA. 

Anomalous  cords,  bands,  or  septa  may  arise  within  the  heart,  and  lead, 
when  sufficiently  pronounced,  to  a  division  of  the  chambers,  and  the 
so-called  double  or  supernumerary  cavities. 

Anomalous  Septa  in  the  Left  Auricle  (Double  Left  Auricle). — Of 
this  interesting  condition  there  are  eight  fully  reported  cases,  by  Church 
(1868),  Fowler,  Martin,  Griffith  (2  cases).  Potter  and  Ransom,  Borst, 
and  Hosch,^  all  of  which  are  summarized  in  an  article  by  the  latter  writer. 
In  these  cases,  a  membranous  diaphragm  perforated  by  one  or  more 
openings  stretches  across  the  left  auricle,  dividing  it  into  a  right  postero- 
superior  chamber,  which  receives  the  pulmonary  veins  and  contains 
the  interauricular  septum,  and  a  left  antero-inferior  chamber,  in  which 
lies  the  auricular  appendix  and  the  mitral  orifice.  The  blood  from  the 
pulmonary  veins  enters  the  large  upper  chamber  and  passes  thence 
through  the  small,  opening  in  the  diaphragm  into  the  lower  chamber 
to  the  mitral  orifice.  When  the  foramen  ovale  is  patent,  a  large  portion 
of  the  pulmonary  blood  may  pass  through  the  open  foramen  into  the 
right  auricle,  thus  depleting  the  greater  circulation  and  throwing  the 
bulk  of  the  work  on  the  right  heart,  which,  as  in  this  case,  becomes  greatly 
hypertrophied,  and  the  left  chambers  very  small. 

Borst  gives  a  careful  anatomical  study  of  his  case,  which  throws  much 
light  on  the  etiology.  In  a  woman,  aged  thirty-eight  years,  dying  with 
failing  compensation,  the  heart  was  of  a  quadrate  shape,  the  right  ven- 
tricle much  hypertrophied  and  dilated,  the  left  auricle  also  greatly  dilated. 
There  was  no  communication  between  the  auricles,  but  the  valvula 
formanis  ovalis  was  absent.  The  left  auricle  was  divided  into  a  large 
upper  cavity  receiving  the  pulmonary  veins  and  a  small  lower  chamber 
containing  the  mitral  orifice  by  a  diaphragm  which  ran  from  above 
anteriorly  and  externally,  downward,  inward  and  backward.  This 
diaphragm  presented  at  its  insertion  below  and  behind,  a  round  hole,  1 
cm.  across,  which  was  the  only  communication  between  the  two  cavities 
through  which  the  blood  from  the  pulmonary  veins  could  be  transmitted 
to  the  mitral  orifice. 

Borst  explained  this  anomalous  diaphragm  as  the  malposed  septum 
primuvi  which  had  been  deflected  to  the  left  in  the  embryonic  heart  by 
a  displacement  of  the  pulmonary  veins  to  the  right,  so  that  they  entered 
the  auricles  between  the  septum  primum  and  secundum,  and  had  thus 
formed  between  them  this  large  secondary  cavity.  The  hole  in  this 
anomalous  septum,  which  communicated  with  the  smaller  cavity  below, 
he  looks  upon  as  the  ostium  secundum  (patent  foramen  ovale). 

Borst's  explanation  receives  corroboration  in  a  remarkable  way  from 
what  may  be  termed  a  complementary  case  reported  by  Sternberg- 

1  Frankf.  Zeitsch.  f.  Path.,  1907,  i,  56. 

2  Verh.  der  XVI  Deut.  path.  Gesell,  1913,  p.  256. 


CONGENITAL  CARDIAC  DISEASE  353 

in  which  three  auricular  chambers  were  present,  separated  from  each 
other  by  strong  septa.  The  left  chamber  received  the  pulmonary  veins 
and  contained  the  mitral  orifice,  and  communicated  with  the  middle 
chamber  by  a  large  ovoid  foramen  (the  ostium  primum  of  Born),  situated 
in  the  lower  portion  of  the  anomalous  septum  (S.  primum)  just  above 
the  mitral  valve.  This  middle  chamber  received  the  superior  cava  only 
and  communicated  with  the  remainder  of  the  right  auricle,  which 
received  the  inferior  cava,  by  a  large  opening.  Sternberg  explained  the 
formation  of  the  middle  chamber  by  a  displacement  to  the  right  of  the 
septum  secundum,  due  to  a  malposition  of  the  inferior  cava,  which 
entered  too  far  to  the  left,  and  pushed  the  septum  secundum  to  the  right, 
just  as  in  Borst's  case  the  anomaly  was  due  to  the  entrance  of  the  pul- 
monary veins  too  far  to  the  right,  so  that  they  cut  off  a  middle  chamber 
by  pushing  the  septum  primum  to  the  left. 

An  anomalous  band  several  cm.  long  and  as  thick  as  a  knitting  needle 
may  traverse  the  left  auricle.  Cases  were  recorded  by  Browicz,  Rolleston 
and  Hosch. 

Anomalous  Septa  in  the  Right  Auricle. — These  are  quite  different 
both  in  structure  and  origin  from  those  in  the  left  auricle.  The  condition 
was  first  described  and  figured  by  Chiari,^  on  the  basis  of  11  original  cases. 
In  all  these  a  system  of  fine  cords,  or  a  reticulum  of  delicate  tissue  identical 
in  structure  with  the  Eustachian  valve  and  attached  to  this  or  to  the 
Thebesian  valve  stretched  across  the  auricle  to  be  attached  to  the  crista 
terminalis  or  auricular  septum  or  other  points.  Similar  structures  in 
the  right  auricle  have  been  recorded,  and  are  to  be  ascribed,  as  Chiari 
pointed  out,  to  a  persistence  and  anomalous  development  of  the  septum 
spurium  or  of  the  right  and  left  valvulse  venosse.  These  are  the  lips  of 
the  opening  of  the  embryonic  sinus  venosus  into  the  right  auricle.  In 
normal  development  they  become  merged  "^dth  the  Eustachian  and 
Thebesian  valves,  and  with  the  septum  secundum.  In  a  case  by  Lesieur 
and  Froment^  of  a  gaping  foramen  ovale  with  an  anomalous  valve  of 
Thebesius,  6  cm.  high,  which  projected  as  a  fenestrated,  incompletely 
attached,  shelf  across  the  right  auricle,  these  authors  ascribe  both  anoma- 
lies to  the  maldevelopment  of  the  valvida  venosa  sinistra,  which  failed 
to  close  in  the  foramen  ovale  by  merging  "^'ith  the  septum  secundum. 
A  connection  between  these  two  anomalies  is  further  established  in 
Ebbinghaus's  case,  in  which  Chiari's  fenestrated  network  extended 
across  the  auricle  from  the  Eustachian  valve,  and  the  interauricular 
septum  showed  fifteen  small  and  two  large  perforations.  Persistent 
left  superior  cava  is  also  frequently  associated. 

Clinical  Aspects.  When  uncomplicated  by  other  defects,  such  septa 
may  exist  without  giving  any  evidence  of  their  presence.  All  the  cases 
of  double  left  auricle  recorded  were  in  adults,  except  that  by  Hosch. 
There  are  certain  dangers,  however.  When  the  septum  is  strongly 
developed,  it  is  liable  to  hamper  the  blood  stream.  Church's  case,  and 
Borst's,  both  died  at  thirty-nine  years  of  failing  compensation,  and  Borst's 
patient   suffered  from   earliest  youth  from  dyspnoea.     Again  the  fine 

1  Ziegler's  Beitr.,  1897,  xxii.,  1.  2  j^y^j^  Medical,  1911 ,  xcvi,  1045. 

VOL.  IV — 23 


354  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

reticulum  that  occurs  in  the  right  auricle  may  supply  a  possible  nidus 
for  thrombotic  processes,  which  may  lead  to  pulmonary  embolism  and 
death.  This  happened  in  Chiari's  first  case,  and  thrombosis  is  related 
also  by  Thilo  and  Thorel. 

Anomalous  Septa  in  the  Ventricles. — These  again  have  a  very  different 
origin  from  those  in  the  auricles.  The  commonest  form  is  a  septum 
shutting  off  the  conus  from  the  sinus  of  the  right  ventricle,  which  probably 
represents  arrest  at  an  early  stage  in  the  development  of  the  heart, 
explained  by  Keith  as  a  "persistence  of  the  lower  bulbar  orifice."  Such 
septa  have  usually  undergone  much  fibrous  thickening,  and  have  been 
ascribed  b}^  many  observers  to  inflammatory  contraction,  but  in  a  case 
reported  by  Bohm  all  evidence  of  fibrosis  was  absent,  and  the  conus 
was  separated  from  the  sinus  by  a  simple  muscular  ridge.  Two  cases 
reported  by  Stephen  Mackenzie^  of  hearts  showing  "three  ventricles," 
separated  by  incomplete  septa,  probably  belong  here. 

In  another  t>^e  of  anomalous  septum,  a  rudimentary  chamber  giving 
off  the  pulmonary  artery,  or  the  (transposed)  aorta  is  cut  off  from  a 
common  ventricle  which  receives  both  auriculoventricular  orifices,  by 
what  appears  to  be  the  malposed  and  defective  interventricular 
septum.  This  t}T)e  is  well  illustrated  by  the  Holmes  specimen  in 
the  jMcGill  Museum  (described  and  figured  under  Triloculate 
Heart,  page  373).  There  are  eight  similar  cases  in  the  literature, 
but  in  all  except  that  of  Holmes  the  aorta  and  pulmonary  artery  were 
transposed. 

Anomalous  cJiordce  tendinece  frequently  cross  the  ventricles  at  irregular 
points.  They  may  consist  of  fibrous  tissue  with  or  without  ordinary 
heart  muscle  fibres,  or  they  may  contain  fibres  from  the  auriculoventricu- 
lar bundle.  These  fibres  have  been  studied  by  Tawara,^  who  gives  a 
review  of  the  literature,  and  by  Monckeberg.^  They  are  explained  by 
the  latter,  as  an  irregular  differentiation  of  the  spongy  musculature  of 
the  embryonic  ventricle,  which  may  or  may  not  contain  Pm-kinje  fibres 
according  to  their  distribution.  Such  abnormal  chordae  may  cause 
loud  musical  murmurs.  A  case  in  point  is  related  by  Hamilton.^  The 
patient  was  a  man  of  forty  with  symptoms  of  aortic  insufficiency,  who 
presented,  in  addition  to  a  soft  diastolic  murmur  with  the  localization 
characteristic  of  this  lesion,  a  second  murmur,  also  diastolic,  but  musical 
in  character,  which  had  its  maximmn  intensity  at  the  third  left  interspace, 
but  was  widely  propagated  over  the  chest,  and  was  so  loud  as  to  be  audible 
two  feet  away  from  the  patient.  The  autopsy,  performed  by  Adami, 
showed  this  musical  murmur  to  have  been  produced  by  an  anomalous 
cord  which  sprang  from  a  small  papillary  muscle  of  its  own,  and  crossed 
the  auricular  surface  of  the  aortic  cusp.  The  aortic  valves  were  thickened 
and  incompetent. 

Anomalous  chordae  passing  from  the  aortic  cusps  to  the  base  of  the 
aorta  (Rohrle),  and  from  pulmonary  cusp  to  base  of  pulmonary  artery 

^  Trans.  Path  Soc.  Lond.,  1880,  xxxi,  63. 

^  Ziegler's  Beitr.,  1906,  xxxix,  563. 

3  Verhand.  XII  Deut.  Path.  Gesell,  1908,  p.  160. 

*  Montreal  Med.  Jour.,  1899,  xxviii,  508. 


CONGENITAL  CARDIAC  DISEASE 


355 


(Poscharissky)  and  from  left  kuricle  to  left  ventricle  through  the  mitral 
orifice,  have  been  recorded. 


Fig.  23 


Aberrant  chordae  tendineae    in    left  ventricle    producing  a  loud    musical    diastolic  murmur.      (From  a 
specimen  in  the  McGiU  Medical  Museum,  presented  by  W.  F.  Hamilton  and  J.  G.  Adami.) 


DEFECTS  OF  THE  INTERAURICULAR  SEPTUM. 


These  may  consist  of  a  simple  patency  of  the  foramen  ovale,  or  true 
defects  of  the  interauricular  septum,  situated  above  or  below  the  foramen, 
and  single  or  multiple,  may  occur.  In  this  series,  the  foramen  ovale 
was  patent  192  times,  and  there  were  46  true  interauricular  defects,  of 
which  15  were  in  its  upper  and  18  in  its  lower  part,  and  13  were  multiple. 
In  14  cases  the  septum  was  rudimentary  and  in  5  it  was  absent  (cor 
biventriloculare) . 

Patent  Foramen  Ovale. — This  orifice,  which  in  the  fetus  is  widely 
open,  allowing  of  the  passage  into  the  systemic  circulation  of  aerated 
placental  blood,  usually  closes  after  birth,  but  its  persistence  in  adult 
life  as  a  valvular  slit  is  so  common  that  this  can  scarcely  be  considered 
abnormal.  Among  711  adults,  Zahn  found  the  foramen  open  in  139, 
and  in  Adami's  records  of  1374  autopsies  at  the  Royal  Victoria  Hospital, 
Montreal,  it  occurred  199  times  (14.5  per  cent.).  From  the  combined 
statistics  of  Bizot,  Ogle,  Klob,  Wallman,  Rostan  and  Hinze,  we  learn 
that  among  2087  hearts  examined,  the  foramen  was  patent  632  times 
(30  per  cent.)  (Herxheimer) . 


356  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

A  widely  patent  foramen  is,  however,  a  true  anomaly,  which,  by 
allowing  free  communication  between  the  auricles,  may  give  rise  to 
various  disturbances.  Of  the  191  cases  in  this  series,  89  were  of  this 
type,  and  of  these,  18  were  instances  of  pure  patency,  unassociated  with 
other  defect,  and  were  therefore  classed  as  the  primary  lesson,  as  affording 
material  for  the  study  of  special  symptoms  and  signs.  In  some  cases 
the  opening  is  very  large,  the  size  of  a  "two-shilling  piece"  (Peacock), 
in  others  it  is  a  "circular  opening  with  thickened  edges  admitting  a 
penhandle,"  and  in  others  again,  it  is  valvular  in  form  but  an  elliptical 
gap  remains  between  the  concave  free  margins  of  the  annulus  ovalis  and 
the  valvula  foraminis  ovalis,  which  are  here  incomplete. 

The  causes  of  patency  may  lie  in  a  rise  of  pressure  in  the  right  chamber 
after  birth,  preventing  the  firm  apposition  of  the  valvula  foraminis 
ovalis  from  the  side  of  the  left  auricle,  and  its  subsequent  closure;  this 
is  the  case  in  the  majority  of  the  cases  complicating  other  defects,  such 
as  pulmonary  stenosis.  Or  the  foramen  may  remain  open  as  a  result 
of  a  true  arrest  of  growth  of  the  primitive  septa,  especially  of  the  septum 
secundum,  an  event  which  may  be  shown  in  the  specimen  by  an  incom- 
plete development,  absence,  or  fenestration  of  the  annulus  ovalis.  The 
association  of  an  anomalous  network  springing  from  the  Thebesian  or 
Eustachian  valve,  with  a  patent  foramen  or  perforated  valvula  foraminis 
ovalis,  and  the  dependence  of  both  anomalies  on  the  persistence  of  the 
embryonic  sinus-valvular  apparatus,  has  been  mentioned.  Persistence 
of  the  left  superior  vena  cava  belongs  to  the  same  complex,  as  is 
illustrated  by  its  occurrence  in  the  case  of  Berthel. 

Such  a  combination  is  well  illustrated  in  a  remarkable  case  in  the 
McGill  Museum  from  the  service  of  C.  F.  Martin.  The  patient  was  a 
woman  of  thirty-eight,  presenting  the  symptoms  of  mitral  stenosis  with 
auricular  fibrillation,  and  a  greatly  dilated  heart.  At  the  autopsy  the 
mitral  valve  was  markedly  stenosed  and  the  foramen  was  widely  patent, 
a  permanent  elliptical  opening  2.5  x  1.5  cm.  large,  with  calcified  lower 
border  existing.  The  auricular  septum  behind  this  was  greatly  thinned 
and  dilated  and  was  bordered  in  the  right  auricle  by  a  flattened  and  much 
fenestrated  ring  representing  the  defective  annulus  ovalis,  and  the 
Thebesian  valve  was  enlarged  and  retiform.  On  the  side  of  the  left 
auricle  at  the  right  posterior  margin  of  the  septum,  there  was  a  curious 
valvular-looking  pocket  2  cm.  deep,  the  free  margins  of  which  were 
attached  to  the  auricular  wall  by  a  plexus  of  tendinous  cords.  At  the 
foot  of  this  pocket  a  channel  could  be  traced  by  a  fine  probe  which  com- 
municated with  the  right  auricle  above  the  Eustachian  valve.  The 
coronary  sinus  and  veins  were  hugely  dilated. 

Defects  in  the  Upper  and  Posterior  Part  of  the  Interauricular  Septum. 
— These  are  extremely  rare.  Two  types  may  be  distinguished,  one  in 
which  the  defect  is  associated  with  an  anomalous  disposition  of  the  great 
veins,  which  is  probably  the  primary  condition,  and  another  in  which 
no  such  associated  anomaly  has  been  demonstrated,  and  which  on  closer 
investigation  may  be  found  to  belong  in  the  same  category  with  the 
first,  or  which  may  require  a  different  explanation.  A  series  of  cases 
has  been  recorded,  in  which  a  large  defect  occupies  the  upper  margin 


CONGENITAL  CARDIAC  DISEASE 


357 


of  the  auricular  septum,  directly  under  the  orifice  of  the  superior  vena 
cava,  which  is  displaced  somewhat  to  the  right,  so  that  its  orifice  looks 
into  both  auricles  through  the  defect.  A  displacement  to  the  right  of 
the  pulmonary  veins,  so  that  these  enter  either  the  superior  vena  cava 
just  before  this  vessel  reaches  the  heart,  or  the  right  auricle  itself,  has 
been  noted  and  is  to  be  considered  the  primary  anomaly.  Paltauf 
thought  that  the  hole  in  the  septum  was  not  a  defect  at  all,  but  represented 
the  orifice  of  the  right  pulmonary  vein,  which  entered  here  directly 
above  the  septum,  with  the  superior  cava.  In  Hepburn's  case  the  wall 
of  the  auricles  was  directly  continuous  with  the  wall  of  these  two  veins, 
which  here  entered  the  auricle  together.  The  foramen  ovale  was  patent 
in  some  cases,  and  is  mentioned  as  closed  in  others. 

Rokitansky  described  7  cases  of  a  similar  anomaly,  in  which  the  inferior 
cava  looked  into  both  auricles  through  the  defect.  In  one  of  these  the 
right  pulmonary  veins  entered  the  right  auricle. 


Fig.  24 


Defect  in  the  upper  part  of  interauricular  septum  (persistent  ostium  secundum),  with  pulmonary 
dilatation,  sclerosing  pulmonary  endocarditis  with  calcification,  and  hypoplasia  of  the  aorta.  From  a 
woman,  aged  sixty-four  years.  Right  chambers  laid  open  to  show:  A,  hypertrophied  and  dilated  right 
auricle;  B,  large  defect  in  the  upper  part  of  interauricular  septum  bounded  below  by  C,  a  stout  muscular 
partition,  the  defective  auricular  septum;  D,  absence  of  annulus  ovaUs  and  of  Eustachian  valve;  E, 
dilated  coronary  sinus  guarded  by  a  defective  valve  of  Thebesius;  F,  entrance  of  superior  vena  cava; 
G,  entrance  of  inferior  vena  cava.  (From  a  specimen  in  the  Medical  Museum^  of  McGill  Univeristy, 
Montreal.     Presented  by  F.  W.  C.  MoFr.    Eeporte*l  by  M.  E.  Abbott  and  J.  Kaufmann.) 


In  a  specimen  in  the  McGill  Museum,  reported  by  Abbott  and  Kauf- 
mann^ (see  Fig.  24),  a  large  ovoid  defect  3  x  3.5  cm.  in  diameter  occupies 

1  Jou7\  Path,  and  Bacteriol.,  1910,  xiv,  525. 


358 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


the  upper  and  back  part  of  the  septum,  but  the  superior  cava  is  not 
displaced,  but  enters  the  right  auricle  in  its  normal  situation  and  is 
separated  from  the  defect  by  a  strong  muscular  cushion.  The  position 
of  the  right  pulmonary  veins  could  not  be  ascertained,  but  the  annulus 
ovalis  and  Eustachian  valve  are  absent,  and  it  is  probable  that  we  are 
here  dealing  with  an  entirely  different  condition,  namely,  a  huge  persistent 
ostium  secundum   (patent  foramen  ovale)   which  has  escaped  closure 


Fig.  25 


Heart  sho-n-ing  (A)  defect  of  interaiiricular  septum  below  (persistent  ostium  primum),  with  (B) 
cleavage  of  right  anterior  segment  of  mitral  valve.  (CO  Interauricular  septum  above  showing  closed 
foramen  ovale.  (D)  Left  posterior  mitral  segment.  From  a  woman,  aged  thirty-two  years,  without 
cardiac  symptoms,  dying  of  perforative  appendicitis.  (From  a  specimen  in  the  Pathological  Museum, 
McGill  University.) 


through  lack  of  development  of  the  secondary  septum.  The  heart 
was  from  a  woman  of  sixty-four  in  good  health  until  the  last  six  years 
of  life,  wdien  cyanosis  began  to  manifest  itself.  The  case  terminated 
with  failing  compensation  and  profound  cyanosis,  and  is  further  remark- 
able in  that  extensive  endarteritic  changes  with  extreme  calcification 
had  taken  place  in  the  pulmonary  valves,  as  the  result  of  the  excess  of 
work  done  by  the  pulmonary  circulation. 


CONGENITAL  CARDIAC  DISEASE  359 

Defects  in  the  Lower  Part  of  the  Interauricular  Septum. — These 
are  somewhat  more  frequent  than  are  defects  at  the  upper  part  of  the 
septum,  but  are  also  rare.  They  are  explained  as  a  persistence  of  the 
ostium  primum,  the  septum  primum  having  failed  to  descend  and  unite 
with  the  cushion  between  the  auriculoventricular  orifice.  Such  a 
defect  has  a  very  characteristic  appearance.  It  lies  directly  above  the 
ventricles,  may  be  of  very  large  size,  and  is  of  a  crescentic  or  semilunar 
shape,  the  thin  border  of  the  auricular  septum,  which  forms  its  upper 
boundary,  arching  across  the  venous  ostia  to  join  the  lower  margin 
formed  by  the  bases  of  the  mitral  and  tricuspid  valves,  which  are  com- 
monly deformed. 

A  common  and  interesting  associated  anomaly  is  a  division  of  the 
anterior  segment  of  the  mitral  valve,  which  is  cleft  from  its  free  border 
up  to  its  insertion,  the  two  parts  converging  here  to  an  acute  angle, 
being  widely  separated  below.  In  five  of  the  seven  cases  of  this  defect 
reported  by  Rokitansky  this  cleavage  occurred.  It  is  well  seen  in  Fig. 
25,  from  a  specimen  in  the  McGill  Museum.  Here  the  foramen  ovale 
was  closed,  as  in  the  cases  reported  by  Griffith,^  Soldner,^  Moore,^  and 
Peacock.'*  It  was  patent  in  the  cases  by  Reineboth,^  Thomson,"  Kilduff  e,^ 
and  Sternberg.^ 

Multiple  Defects. — The  valvula  foraminis  ovalis  is  not  infrequently 
perforated  by  numerous  small  openings,  as  in  a  specimen  (Fig.  26)  in 
the  McGill  Museum,  and  in  several  cases  in  this  series.  This  recalls 
the  fenestrated  septum  seen  in  birds,  and  suggests  an  arrest  of  develop- 
ment at  this  stage.  In  a  case  reported  by  Dublitzhaja,  of  combined 
defects  of  the  auricular  and  ventricular  septa,  the  auricular  septum, 
defective  below,  "hung  like  a  curtain  over  the  common  ventricle,"  there 
was  a  large  patent  foramen  ovale,  and  also  a  large  hole  at  the  upper  and 
posterior  border  of  the  valvula  foraminis  ovalis.  An  almost  identical 
case  is  reported  by  Ebbinghaus.  In  a  man  of  fifty-two,  there  were  fifteen 
small  perforations  and  two  large  holes  at  either  border,  representing  the 
persistent  ostium  primum  and  secundum  (patent  foramen  ovale),  and 
the  right  auricle  contained  an  anomalous  network. 

Aneurismal  Pouching. — Aneurismal  pouching  of  the  fossa  ovalis 
is  sometimes  seen  (Figs.  26  and  27).  In  most  cases  the  convexity  of  the 
pouch  is  toward  the  left  auricle,  indicating  a  rise  of  pressure  in  the  right 
heart  before  death. 

Secondary  Pathological  Changes. — -The  alterations  in  the  circulation 
produced  by  large  defects  in  the  interauricular  septum  induce  various 
secondary  results.  Hypoplasia  of  the  aorta  and  a  corresponding  dilata- 
tion of  the  pulmonary  artery  are  common,  and  the  latter  vessel  may  also 
be  atheromatous  from  excessive  strain.  Hypertrophy  and  dilatation  of 
the  heart  are  the  rule,  and  this  may  be  confined  to  the  right  chambers 

1  Manch.  Med.  Chron.,  1902,  iv,  383.      _  ^  Munich  Thesis,  1904. 

^  Trans.  Path.  Soc,  London,  1881,  xxxii,  37. 

4  Ibid.,  1847,  i,  61. 

5  Deut.  med.  Woch.,  1895,  xxi,  870.  «  Proc.  Anat.  Soc,  1902-3,  xxxvi. 

7  Am.  Jour.  Med.  Sc,  1914,  cxlvii,  880. 

8  XVI  Verh.  d.  Deut.  GeselL,  1913,  p.  253 


360 


DISEASES  OF  THE  CIRCULATORY  SYSTEM 


Fig.  26 


Fenestrated  membrane  bulging  into  fossa  ovalis.     (From  a  specimen  in  tlie  Fatliological 
Museum,   McGill  University.) 


Fig.  27 


Bulging  of  fossa  ovalis  into  left  auriclo.    Valvular  patent  foramen  ovale.     (From  a  specimen  in  the 
McGill  Pathological  Museum.) 


CONGENITAL  CARDIAC  DISEASE  361 

or  may  be  generalized,  the  changes  in  the  right  side  almost  always  pre- 
ponderating. In  none  of  the  cases  in  this  series  was  the  left  ventricle 
hypertrophied  in  excess  of  the  right.  Both  auricles  are  usually  much 
dilated  and  in  four  of  our  cases  this  took  place  without  any  increase  in 
size  of  the  ventricles.  Congestive  changes  and  oedema  of  the  lungs, 
enlargement  of  the  liver,  and  passive  congestion  of  all  organs  usually 
occur  as  late  results  of  the  overloading  of  the  pulmonary  circulation. 

Symptoms  and  Signs. — Large  defects  in  the  interauricular  septum 
may  exist  Vv'ithout  giving  any  sign  or  symptom  of  their  presence  and 
without  interfering  with  the  duration  of  life  of  the  individual.  The 
characteristic  feature  of  the  majority  of  the  cases  may  be  summed  up 
as  an  absence  or  but  very  slight  manifestation  of  cyanosis,  in  the  presence 
of  distinctive  physical  signs  of  the  defect.  In  a  few  rare  instances, 
symptoms  of  congenital  cyanosis  have  set  in,  in  cases  of  uncomplicated 
foramen  ovale  in  very  early  life.  There  are  three  such  in  our  series, 
one  by  Johnson,  of  a  man  aged  twenty-seven  with  moderate  cyanosis 
and  slight  clubbing,  and  the  others  by  Simmons^  and  Foster^  in  infants, 
showing  cyanosis  and  dyspnoeic  attacks. 

The  above  statement  is  especially  true  of  defects  at  the  upper  part 
of  the  septum.  All  the  patients  in  this  series  reached  middle  life  without 
giving  any  evidence  of  disease,  and  with  the  exception  of  two  cases, 
all  died  of  independent  conditions  unassociated  with  the  defect.  In 
Greenfield's  case,  a  man,  aged  fifty-three  years,  failing  compensation 
set  in  eighteen  months  before  death  and  cyanosis  was  marked  at  the  close. 

Not  infrequently  symptoms  first  develop  after  some  event,  such  as  an 
intercurrent  pneumonia,  has  embarrassed  the  pulmonary  circulation, 
and  thus  caused  a  rise  of  pressure  in  the  right  auricle,  followed  by  a 
passage  of  venous  blood  from  right  to  left  through  the  defect.  The 
picture  is  usually  that  of  a  relative  mitral  insufficiency,  with  marked 
cyanosis  supervening  as  a  terminal  event.  These  are  the  cases  of  la 
cyanose  tardive  described  by  Bard  and  Curtillet  as  characteristic  of 
auricular  septal  defects.  The  hypoplasia  of  the  aorta  so  often  associated 
with  patent  foramen  probably  assists  in  the  development  of  failing 
compensation,  and  therefore  forms  an  essential  part  of  the  picture. 

Auricular  septal  defects  are  not  infrequently  associated  with  mitral 
stenosis.  Such  cases,  of  which  there  are  seven  in  our  series,  usually 
run  their  course  under  the  guise  of  the  complicating  mitral  condition, 
the  defect  probably  adding  a  factor  in  the  development  of  the  cardiac 
distress.  An  example  of  the  absence  of  characteristic  symptoms  in 
extensive  communication  between  the  auricles  is  seen  in  Dr.  Martin's 
case  of  mitral  stenosis  with  open  foramen.  The  clinical  picture  here  was 
that  of  the  terminal  stages  of  uncomplicated  mitral  stenosis,  and  the 
degree  of  cyanosis  was  not  greater  than  might  have  been  expected  in 
such  a  condition.  A  presystolic  thrill  with  maximum  intensity  at  the 
left  fourth  interspace,  and  a  presystolic  murmur  audible  towards  the 
base  which  disappeared  as  the  end  approached,  were  the  only  evidence 
of  the  defect. 

1  Inter.  Med.  Jour.  Austr.,  Feb.  20,  1906. 

^  Dublin  Quarterly  Jour.  Med.,  1863,  xxxvi,  112. 


362  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

Pallor  of  the  surface  is  a  common  characteristic  (Mouls^  as  is  also  a 
certain  delicacy  of  build,  the  result  evidently  of  the  diminished  amount 
of  blood  in  the  systemic  circulation.  Dyspnoea  and  tachycardia,  bron- 
chitis and  signs  of  congestive  changes  in  the  lungs  and  of  passive  conges- 
tion in  the  viscera  and  enlargement  of  the  liver  are  also  common,  from 
excess  of  blood  entering  the  right  heart.  When,  in  adults  with  these 
symptoms,  and  with  cyanosis  slight,  absent,  or  of  extremely  late  appear- 
ance, we  find  distinctive  physical  signs  of  a  defect  localized  over  the 
upper  and  middle  thirds  of  the  heart,  we  may  feel  fairly  sure  that  we 
are  dealing  with  an  auricular  septal  defect.  A  correct  diagnosis  was 
made  on  this  basis  in  a  patient  at  the  Royal  Victoria  Hospital  whose 
heart  is  in  the  McGill  Museum.  In  a  woman  of  twenty-eight  years, 
of  somewhat  infantile  appearance,  in  whom  cyanosis  was  entirely  absent, 
a  loud  harsh  systolic  murmur  with  maximum  intensity  at  the  third  left 
interspace,  4.5  cm.  from  the  midsternum,  was  heard  loudly  all  over  the 
front  of  the  chest  and  over  the  left  back,  the  cardiac  dulness  was  greatly 
increased  in  all  directions,  and  there  was  a  marked  systolic  thrill  at  the 
base  to  the  left  of  the  midsternum,  extending  downward  to  the  top  of 
the  fourth  rib.  The  neck  veins  were  much  distended,  but  no  positive 
venous  pulse  was  detected.  Death  occurred  from  chronic  interstitial 
nephritis.  The  autopsy  showed  a  large  patent  foramen  ovale  admitting 
the  thumb,  with  cribriform  perforation  of  the  valvula  foraminis  ovalis, 
hypoplasia  and  slight  coarctation  of  the  aorta,  and  infantile  genitalia. 

The  slight  degree  of  cyanosis  that  characterizes  these  cases  and  the 
effect  of  the  lesion  upon  the  duration  of  life  are  well  shown  by  the 
analysis  of  the  43  cases  of  auricular  septal  defects  classified  as  the 
primary  lesion  in  this  series.  Cyanosis  was  entirely  abse?it  in  21 
cases.  In  2  of  these  death  apparently  due  to  the  defect  occurred 
in  4,  in  2  cases  from  failing  compensation,  in  1  from  sudden 
collapse,  and  in  1  (Popper^)  by  sudden  development  of  oedema  and 
dyspnoea.  Terminal  cyanosis  occurred  in  13  cases  of  the  series.  The 
ages  of  the  patients  ranged  from  ten  to  sixty-four  years,  and  death 
ocurred  from  pneumonia  in  2,  from  failing  compensation  in  6,  and  from 
cerebral  hemorrhage  in  1  (Ebbinghaus) .  In  4  other  cases  of  the  43, 
cyanosis  was  described  as  slight  and  in  3  only  as  moderate  (Johnson, 
Foster,  Simmons). 

Physical  signs  are  sometimes  absent,  but,  in  widely  patent  foramen 
ovale,  and  in  defects  at  the  lower  part  of  the  septum,  a  sufficiently  char- 
acteristic murmur  is  usually  present,  and  sometimes  a  corresponding 
precordial  thrill.  In  defects  at  the  lower  part  of  the  septum  in  which  ex- 
tensive deformities  of  the  auriculoventricular  cusps  are  usually  associated, 
the  murmur  produced  is  often  heard  best  at  the  apex,  and  is  "confused," 
or  "  roaring"  in  character.  It  was  associated  in  4  out  of  10  cases  analyzed 
with  a  thrill  continuous  or  systolic  or  "presystolic"  in  rhythm.  In 
widely  patent  foramen,  the  murmur  is  usually  systolic  or  presystolic 
and  is  sometimes  soft,  but  often  harsh  and  rasping,  or  of  a  peculiar 
blowing,  or  even  musical  character,  and  may  be  localized  in  the  second, 

1  Rev.  Mens,  des  Mai.  des  Enfant,  1888,  vi,  p.  151. 

2  Wien.  klin.  Woch.,  1909,  No.  16,  p.  562. 


CONGENITAL  CARDIAC  DISEASE  363 

third  or  fourth  mterspace  near  the  left  sternal  border,  or  over  the  mid- 
sternum  in  this  situation,  or  may  be  diffusely  heard  over  the  precordium. 
It  is  frequenUy  audible  in  the  left  back,  and  may  be  transmitted  to  the  apex 
and  axilla,  or  (occasionally)  to  the  left  subclavicular  region  (Markham's'- 
case),  or  may  be  heard  loudly  over  the  whole  chest.  Among  the  18 
cases  of  patent  foramen  analyzed,  a  murmur  of  the  above  character 
was  noted  in  14.  In  9  it  was  systolic,  in  3  presystolic,  in  2  it  varied 
from  presystolic  to  systolic,  and  in  1  only  (Bard  and  Curtillet)  it  was 
diastolic  in  rhythm.  In  3  of  these  cases  the  murmur  was  associated 
with  a  thrill  of  corresponding  localization  and  rhythm. 

Both  murmur  and  thrill  may  vary  in  intensity  and  in  rhythm  with  the 
position  of  the  patient  being  noted  as  presystolic  or  systolic  on  different 
examinations.     This  inconstancy  may  be  a  useful  diagnostic  point  (Ohm). 

When  an  organic  mitral  insufficiency  is  combined  with  a  patent  foramen, 
the  regurgitation  of  blood  through  the  defect  into  the  right  auricle  during 
systole  of  the  ventricles  may  give  rise  to  a  positive  venous  pulse  in  the 
neck,  without  the  presence  of  tricuspid  insufficiency.  Cases  are  reported, 
confirmed  by  autopsy,  in  which  the  tricuspid  was  healthy,  and  in  which 
no  signs  of  tricuspid  regurgitation  existed  during  life.  When  mitral 
stenosis  is  present  this  sign  is  of  no  value,  as  it  is  likely  to  be  due  to  the 
auricular  fibrillation  so  frequent  in  this  condition. 

Paradoxical  Embolism. — A  serious  clinical  significance  is  given  to 
patent  foramen  ovale  by  the  fact  that  particles  may  be  carried  through 
the  defect  from  the  venous  circulation  to  the  arteries  of  the  brain,  or 
from  the  systemic  arteries  to  the  lung,  leading  to  instant  death.  This 
possibility  was  first  pointed  out  by  Cohnheim  in  an  observation  of  a 
woman  dying  of  embolism  of  the  middle  cerebral  artery.  The  foramen 
admitted  three  fingers,  the  arterial  system  was  clear,  while  the  primary 
thrombi  lay  in  the  veins  of  the  lower  extremities.  Ohm^  collected  11 
such  cases  from  the  literature  in  which  an  embolus  from  a  thrombosed 
vessel  or  a  metastasis  from  a  new  growth  undoubtedly  passed  through 
the  open  foramen  and  added  an  original  case.  Ballet^  collected  6  cases 
of  death  from  cerebral  abscess  from  infected  emboli  in  which  both  cardiac 
and  cerebral  symptoms  were  present  during  life.  In  3  of  these  there 
was  a  patent  foramen,  in  2  a  defect  of  the  interventricular  septum. 
Verse*  described  2  typical  cases. 

DEFECTS  OF  THE  INTERVENTRICULAR  SEPTUM. 

The  interventricular  septum  may  be  completely  absent  (cor  biatriatrum 
triloculare),  or  it  may  be  rudimentary,  represented  by  a  falciform  process 
growing  up  from  the  lower  and  anterior  wall  of  the  ventricle,  or  localized 
defects  may  occur.  These  usually  lie  at  its  base,  and  are  relatively 
common  in  association  with  other  anomalies,  but  are  not  frequent  alone. 
Defects  elsewhere  than  at  the  base,  whether  alone  or  in  combination, 
are  among  the  rarest  of  cardiac  anomalies. 

1  Trans.  Path.  Soc,  London,  1881,  viii,  142. 

2  Zeit.f.  klin.  Med.,  1907,  Ixii,  374.  3  Archiv.  gen.  de.  med.,  1880. 
*  XIII  Verh.  Deut.  Path.  Gesell,  1909,  p.  215. 


364  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

Defects  at  the  Base. — The  cases  of  congenital  cardiac  disease  here 
studied  have  been  drawn  only  from  reliable  sources,  and  all  have  post- 
mortem reports  attached,  which  should  make  them  a  fair  index  of  rela- 
tive frequency.  It  is  therefore  of  interest  to  note  that  among  them, 
while  defects  elsewhere  in  the  septum  are  exceedingly  rare,  "pure" 
defects  at  the  base  are  commoner  than  is  usually  supposed,  and,  in 
combination  with  other  defects  they  rank  as  the  most  frequent  cardiac 
anomaly. 

Among  the  631  cases,  a  defect  at  the  base  occurred  177  times,  else- 
where than  at  the  base,  12  times,  making  189  cases,  or  30  per  cent.  Of 
the  177  defects  at  the  base,  34  were  classed  as  the  primary  defect  and 
143  complicated  other  conditions.  Of  the  34  "primary"  defects  at  the 
base,  8  were  combined  with  rechtslage  of  the  aorta,  and  in  1  of  these 
there  was  also  pulmonary  hypoplasia;  in  another  there  was  pulmonary 
hypoplasia  without  rechtslage;  in  3  there  was  a  patent  ductus  arteriosus, 
leaving  22  "pure"  defects  at  the  base  unassociated  with  rechtslage  or 
other  anomaly  except  (in  5  cases)  bicuspid  or  defective  aortic  or  pul- 
monary valves 

Of  the  143  defects  at  the  base  complicating  other  anomalies,  75  were 
in  cases  of  pulmonary  stenosis  or  atresia,  in  53  of  which  there  was  also 
rechtslage  of  the  aorta.  Of  the  remainder,  26  were  in  transposition  of  the 
great  trunks,  3  in  other  defects  complicated  by  rechtslage,  and  13  in  per- 
sistent truncus  arteriosus.  That  is  to  say,  in  42  other  cases  the  defect 
was  associated  with  an  irregularity  of  development  of  the  great  trunks. 
In  the  remaining  26  of  these  143  cases,  the  septal  defect  was  associated 
with  tricuspid  atresia  in  9  cases,  with  mitral  atresia  in  1,  with  aortic 
stenosis  or  dextrocardia  in  2,  and  with  partial  defect  of  the  aortic  septum 
in  two. 

Pathogenesis. — The  combination  of  a  defect  of  the  interventricular 
septum  vvith  pulmonary  stenosis  and  rechtslage  of  the  aorta  constitutes 
one  of  the  commonest  forms  of  congenital  cardiac  disease.  So  frequent 
is  the  combination  that  a  causal  connection  between  the  three  conditions 
has  been  sought,  and  rival  theories  as  to  which  is  the  primary  lesion  have 
been  suggested. 

Much  light  was  thrown  upon  this  subject  by  Rokitansky,  who  con- 
cluded that  non-inflammatory  pulmonary  stenosis,  displacement  to 
the  right  of  the  aorta,  transposition  of  the  great  arterial  trunks,  and 
defects  at  the  base  Jf  the  interventricular  septum  were  alike  dependent 
upon  a  common  cause,  a  deviation  of  the  aortic,  so  that  it  failed  to  unite 
with  the  interventricular  septum.  He  divided  the  latter  into  a  part 
anterior,  and  one  posterior  to  the  undefended  space,  and  classified  defects 
at  the  base  according  as  they  lay  in  the  anterior  part  of  the  anterior 
septum,  in  the  posterior  part  of  the  anterior  septum,  or  in  the  posterior 
septum.  He  pointed  out  that  their  usual  situation  was  in  the  "posterior 
part  of  the  anterior  septum,"  that  is,  just  anterior  to  the  pars  mem- 
branacea,  in  which  case  he  believed  they  were  practically  invariably 
associated  with  a  malposition  of  the  arterial  trunks.  Keith  has  suggested 
that  in  the  large  number  of  cases  in  which  a  septal  defect  is  associated 
with  stenosis  of  the  conus  of  the  right  ventricle  the  defect  is  a  direct 


CONGENITAL  CARDIAC  DISEASE 


36.367 


Fig.  28 


result  of  the  inadequate  expansion  of  the  bulbus  cordis  to  form  the 
infundibuhim  of  the  right  ventricle. 

Later  advances  in  embryological  knowledge  show  that  the  aortic 
septum  is  prolonged  downward  to  assist  in  closing  the  interventricular 
septum  at  the  undefended  space,  instead  of  the  interventricular  septum 
growing  upward  to  form  part  of  the  aortic  wall,  as  Rokitansky  supposed. 
Moreover,  independent  defects  of  the  interventricular  septum  in  this 
situation,  unassociated  with  any  alterations  in  the  relations  of  the  great 
arterial  trunks,  and  evidently  not  of  inflammatory  origin,  may  and  do 
occur.  This  is  so  in  the  specimen  seen  in  Fig.  28,  and  in  cases  reported 
by  Orth,^  Arnold,  Preisz,^  and  Hart.^ 
Such  conditions  cannot  be  explained 
on  Rokitansky's  theory  as  due  to  de- 
viation of  the  septum,  or,  a  deficient 
expansion  of  the  infundibuhim,  but 
are  due,  as  Keith  himself  points 
out,  to  a  primary  arrest  of  growth 
of  unknown  origin.  In  Hart's  case 
an  interesting  associated  anomaly, 
which  may  have  had  some  bearing 
on  the  defect,  was  an  anomalous  cord 
which  extended  from  the  lower  border 
of  the  conus  of  the  right  ventricle 
through  the  septal  opening,  to  the 
anterior  segment  of  the  mitral  valve. 

Pathology. — The  commonest  situ- 
ation for  the  defect  is  directly  beneath 
the  aortic  cusps  and  just  anterior  to 
the  undefended  space  (Rokitansky's 
posterior  part  of  the  anterior  sep- 
tum) (Fig.  28).  Here  it  lies  with  the 
fleshy  muscular  septum  before  it  and 
the  thin  pars  membranacea  behind, 

and  opens  in  the  right  heart  beneath  the  septal  cusp  of  the  tricuspid, 
sometimes  perforating  this  or  bulging  the  (adherent)  tricuspid  leaflet 
before  it,  or  opening  into  the  right  auricle  directly  above  the  base  of  the 
tricuspid,  thus  establishing  a  communication  between  this  cavity  and 
the  two  ventricles.  IMore  rarely  the  defect  is  placed  farther  forward 
in  the  septum  in  its  anterior  fleshy  part,  just  behind  the  front  wall  of  the 
heart,  and  is  separated  behind  from  the  undefended  space  by  a  muscular 
column,  opening  into  the  conus  of  the  right  ventricle  below  the  pul- 
monary valves.  (Rokitansky's  anterior  part  of  the  anterior  septum). 
Examples  are  the  cases  by  Coupland^  and  Rolleston.^  Keith  points 
out  that  in  these  cases,  the  defect  is  evidently  in  the  musculature  of  the 
interbulbar  septum,  i.  e.,  it  is  in  that  part  of  the  interventricular  septum 


Defect  of  the  interventricular  septum  at 
undefended  space.  Heart  of  infant.  No 
other  anomaly.  (From  a  specimen  in  the 
McGill  Pathological  Museum.) 


1  Virchoivs  Archiv,  1880,  Bd.  82,  529. 
3  Virchows  Archiv,  1905,  Bd.  181,  p.  7.3. 
*  Trans.  Path.  Soc.  London,  1879,  xxx,  226. 
=  lUd.,  1891,  xlii,  65. 


2  Ziegler's  Beitrage,  1890,  Bd.  7,  245. 


^j66  diseases  of  the  circulatory  system 

which  in  the  embryo  formed  the  proximal  part  of  the  bulbus  cordis 
before  its  division  into  the  conus  of  the  pulmonary  artery  and  the  vesti- 
bule of  the  aorta. 

The  defect  varies  in  form  and  size  from  a  pinhead  perforation  with 
tendinous  edges,  a  round  or  oval  hole  admitting  a  goose-quill,  knitting 
needle,  index  finger,  etc.,  to  a  large  triangular,  semilunar,  or  crescentic 
space  with  thick-walled  lower  muscular  border.  Aneurismal  pouching 
of  the  pars  membranacea  into  the  right  ventricle  may  occur,  with  multiple 
sacculations  perforated  at  their  apices  at  one  or  more  points  (vide  infra) . 

Quite  frequently  the  margins  of  the  defect  with  the  adjacent  valves 
are  the  seat  of  an  acute  inflammatory  process  which  apparently  originates 
at  this  point,  because,  as  the  seat  of  greatest  strain,' it  affords  a  site  of 
lowered  resistance.  Such  a  case  is  reported  by  Gordon.^  In  a  boy 
aged  five  years  who  died  of  malignant  endocarditis,  a  marked  precordial 
thrill,  and  loud,  harsh  systolic  murmur  over  the  whole  chest,  with  maxi- 
mum intensity  at  the  third  and  fourth  left  cartilages,  indicated  the  septal 
defect.  The  autopsy  showed  a  congenital  opening,  admitting  a  lead 
pencil,  between  the  ventricles.  The  pulmonary  valves  and  adjacent 
M^all  of  the  right  ventricle  were  the  seat  of  a  number  of  large  grayish- 
green  vegetations  which  extended  below  the  level  of  the  interventricular 
opening. 

Moschcowitz^  related  a  very  similar  finding  in  a  woman  of  twenty-nine 
years,  who  had  had  cardiac  symptoms,  palpitation,  dyspnoea  and  occa- 
sional oedema  of  the  legs  since  her  fifteenth  year,  with  exacerbation  dur- 
ing the  last  four  months,  and  symptoms  of  acute  infection  with  chills  and 
septic  fever  for  one  week.  The  blood  cultures  on  one  occasion  showed 
Streptococcus  viridans.  At  the  autopsy  both  ventricles  were  hypertrophied, 
and  the  right  also  dilated,  and  there  was  a  defect  in  the  membranous 
septum  admitting  a  lead-pencil.  The  pulmonary  valves  were  replaced 
by  large  gray  pedunculated  vegetations,  which  extended  up  the  wall  of 
the  pulmonary  artery  to  its  bifurcation,  and  had  led  to  multiple  emboli 
in  either  lung.  The  prolonged  history  of  cardiac  symptoms  without 
adequate  cause  suggested  a  congenital  lesion  upon  which  the  malignant 
endocarditis  had  been  engrafted. 

Dr.  W.  Thalhimer  has  sent  me  photographs  of  a  heart  from  a  cyanotic 
boy  of  nine  years,  with  a  huge  septal  defect  and  pulmonary  stenosis  in 
which  a  vegetative  process  had  developed  along  the  line  of  closure  of  the 
mitral  and  tricuspid  valves.  As  there  was  no  clinical  history  of  an  infec- 
tive process,  no  Aschoff  bodies  in  the  myocardium,  and  no  evidences  of 
bacteria  in  the  vegetations  on  microscopic  examination,  he  is  inclined 
to  explain  the  vegetations  on  the  ground  of  mechanical  strain  by  the 
unnatural  whirls  in  the  blood  stream,  which  have  caused  microscopic 
injuries  of  the  valves  and  have  thus  given  rise  to  an  aseptic  thrombosis. 
From  a  statistical  study  of  the  literature  one  is  led  to  conclude  that,  at 
least  in  a  large  proportion  of  cases,  the  infective  nature  of  the  inflam- 
matory process,  so  common  in  the  neighborhood  of  cardiac  defects, 
has  been  demonstrated. 

1  Brit.  Med.  Journ.,  1897,  ii,  1174.        ^  pj-^c.  New  York  Path.  Soc,  1914,  xiv,  18. 


CONGENITAL  CARDIAC  DISEASE  367 

In  a  case  reported  by  Hebb^  of  a  girl,  aged  eighteen  years,  whose  heart 
showed  a  funnel-shaped  defect  at  the  base  of  the  septum  admitting  a 
goose-quill,  there  were  large  vegetations  bn  the  aortic,  mitral,  and  pulmo- 
nary valves,  and  a  'patch  of  vegetations  ivas  also  situated  on  the  wall  of  the 
right  ventricle  opposite  the  defect.  This  observation,  which  is  repeated 
in  several  other  cases,  as  well  as  the  frequent  localization  of  the  vegeta- 
tions in  the  right  ventricle  affords  an  interesting  proof  of  the  fact  that 
under  normal  conditions  the  current  of  blood  flows  from  the  left  ventricle 
to  the  right  through  the  defect.  It  is  natural  to  suppose  that  this  would 
be  so,  for  the  pressure  in  the  systemic  circulation  is  normally  higher 
than  it  is  in  the  pulmonary.  Further  anatomical  confirmation  of  this 
direction  of  the  stream  is  afforded  by  the  oblique  direction  and  funnel- 
shape,  with  its  larger  end  toward  the  left  ventricle  which  the  opening 
often  assumes,  and  also  by  the  not  infrequent  occurrence  of  patches  of 
fibrosis  on  the  opposite  wall  of  the  right  ventricle. 

Septal  defects  may  exist  without  producing  any  change  in  the  heart 
chambers,  but  they  lead,  still  more  frequently  than  do  defects  of  the 
interauricular  septum,  to  hypertrophy  and  dilatation  of  both  ventricles. 
Where  the  defect  is  combined  with  rechtslage  of  the  aorta,  marked  hyper- 
trophy of  the  right  ventricle  is  a  constant  feature.  The  pulmonary 
artery  may  be  markedly  dilated,  as  in  9  cases  of  the  34  "primary"  defects 
at  the  base. 

The  distribution  of  the  auriculoventricular  junctional  bundle  in 
septal  defects  is  of  much  interest,  and  has  been  investigated  by  Keith, 
Monckeberg,  Morison,^  and  others.  As  is  well  known  this  bundle 
emerges  from  the  auriculoventricular  node  close  to  the  interauricular 
septum  behind  the  medial  cusp  of  the  tricuspid  valve,  and  divides  into 
two  branches,  the  left  of  which  pierces  the  interventricular  septum  just 
in  front  of  the  pars  membranacea,  and  passes  downward  superficially 
beneath  the  endocardium  of  the  septum  to  be  distributed  to  the  papillary 
muscles  and  columnee  carnese  of  this  chamber,  while  the  right  branch 
runs,  more  deeply  imbedded  in  the  musculature  of  the  right  side  of  the 
septum,  to  the  apex  of  the  right  ventricle.  In  most  of  the  cases  of  septal 
defect  examined  there  was  surprisingly  little  change  in  this  normal 
arrangement,  the  fibres  streaming  over  the  free  border  of  the  septum 
that  formed  the  base  of  the  defect  toward  the  apices  of  their  respective 
ventricles.  In  Morison's  case  the  left  branch  was  abortive.  Keith 
described  an  abnormal  band  of  "subaortic  musculature,"  which  may 
develop  in  the  pars  membranacea  and  overlie  the  bundle  as  it  courses 
down  the  surface  of  the  septum  in  the  left  ventricle,  and  in  one  of  Moncke- 
berg's  cases  of  septal  defect  the  bundle  lay  deeply  in  the  musculature 
of  the  left  septum  instead  of  sub-endocardially  as  normally  occurs. 
Recent  investigations  by  Flack  and  Mall  have  shown  that  the  inter- 
ventricular septum  is  formed,  not  by  a  process  growing  upward  from 
below,  as  Rokitansky  supposed,  but  by  a  hollowing  out  of  the  spongy 
musculature  of  the  embryonic  ventricle  to  form  the  right  and  left 
chambers;  the  tip  of  the  inferior  septum,  therefore,  represents  the  wall 

1  Trans.  Path.  Soc.  London,  1897,  xlviii,  41. 

2  Jour.  Anat.  and  Physiol.,  1913,  xlvii,  459. 


368  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

of  the  lumen  of  the  original  cardiac  tube,  and  this  may  account  for  the 
persistence  of  the  bundle  at  this  point  in  septal  defects. 

Symptoms  and  Physical  Signs  of  Septal  Defects. — These  may  be  absent 
and  the  defect  discovered  at  autopsy.  When  cyanosis  is  present  it  may 
be  slight  or  transient,  appearing  only  on  exertion,  or  terminal  at  the  end 
of  a  long  life,  or,  more  rarely,  and  usually  in  those  cases  associated  with 
rechtslage  of  the  aorta,  it  may  be  well  marked.  Among  the  34  "primary" 
defects  at  the  base  there  was  an  entire  absence  of  cyanosis  in  17;  it  was 
slight  in  5,  moderate  in  2,  marked  in  3,  and  terminal  in  4  cases. 

Physical  signs  may  also  be  absent,  as  in  two  otherwise  normal  hearts 
with  defects  admitting  a  goose-quill,  in  the  McGill  Museum.  On  the 
other  hand,  physical  signs  atypical  of  acquired  valvular  disease  are 
often  present,  even  in  the  absence  of  symptoms,  and  are  frequently 
sufficiently  characteristic  to  permit  of  a  diagnosis  being  made.  In 
pronounced  cases  there  may  be  visible  pulsation  and  precordial  bulging, 
and  a  thrill,  usually  systolic  in  time  and  diffuse  over  the  precordium, 
or  most  marked  over  the  middle  of  the  cardia,  is  common  and  is  more 
frequent  than  in  auricular  septal  defects.  A  thrill  was  present  in  no 
less  than  11  of  the  34  cases  of  primary  defects  at  the  base;  in  10  being 
systolic  and  in  1  a  "continuous  vibration."  In  7  it  was  diffused  over 
the  whole  cardia;  in  3  it  was  most  marked  over  its  upper  half;  in  2  others 
it  was  localized  at  the  apex. 

A  harsh,  systolic  murmur  localized  in  the  third  or  fourth  left  space 
is  the  most  frequent  evidence  of  the  defect.  Sometimes  a  very  small 
hole  may  be  accompanied  by  a  very  loud  murmur.  Roger  described 
as  characteristic  a  "  single  long,  constant  murmur  beginning  with  systole 
and  continuing  through  both  heart  sounds,  localized  in  the  upper  third 
of  the  precordial  region."  Reiss  mentions  as  typical  a  loud  systolic 
murmur  in  the  middle  of  the  precordium,  localized  over  the  inner  part 
of  the  third  left  space  and  the  fourth  rib. 

A  murmur  apparently  due  to  the  defect  was  present  in  26  of  the  34 
cases.  In  those  in  which  its  character  was  specified  it  was  loud  in  18, 
rough  in  3,  whistling,  grating,  harsh,  sawing,  each  in  1,  rasping  in  3;  in  3 
instances  it  was  blowing.  It  was  systolic  in  rhythm  in  all  26  cases,  and 
in  3  of  these  a  diastolic  murmur  was  present  as  well.  The  point  of 
maximum  intensity  was  12  times  in  the  upper  third  of  the  precordium  near 
the  left  sternal  border;  of  these,  in  4  it  was  stated  to  be  at  the  third  left 
space,  in  2  others  also  at  the  fourth  left  space,  in  2  at  the  third  costal 
cartilage  (in  1  of  which  it  was  heard  with  equal  intensity  at  the  apex), 
in  2  at  the  pulmonary  cartilage  and  second  left  space,  and  in  1  "  over  the 
middle  of  the  sternum  opposite  the  third  left  interspace."  Besides  these, 
in  2  other  cases  it  was  "along  the  left  sternal  border,"  "just  to  the  left 
of  the  xiphoid  cartilage,"  and  "at  the  aortic  cartilage;"  in  2  (in  1  of 
which  it  was  associated  with  rechtslage)  it  was  "loudest  at  the  apex," 
and  in  4  it  was  diffuse  over  the  precordium. 

The  murmur  is  usually  transmitted  downward  along  the  left  sternal 
border,  and  is  frequently  heard  behind  in  the  left  infrascapular  region. 
It  may  be  diffused  over  the  whole  precordium,  but  is  usually  not  heard 
in  the  axilla  nor  below  the  clavicle.     In  6  cases  in  this  series  it  was  so 


CONGENITAL  CARDIAC  DISEASE  369 

loud  as  to  be  heard  over  the  whole  chest,  and  in  2  cases  it  could  be 
traced  into  the  vessels  of  the  neck. 

Eisenmenger^  claimed  that  a  systolic  murmur  produced  by  the  defect 
may  be  transmitted  along  the  aorta,  and  thus  be  heard  in  the  vessels 
of  the  neck,  when  pulmonary  hjq^oplasia  is  also  present,  in  which 
case  the  blood  is  diverted  by  the  obstruction  from  its  usual  course  into 
this  vessel,  and  so  passes  from  right  to  left  into  the  aorta.  In  his  own 
case,  diagnosed  by  Schrotter  before  death,  there  was  a  defect  admitting 
the  thumb  in  the  posterior  part  of  the  anterior  septum,  with  rechtslage 
of  the  aorta  and  a  dilated  pulmonary  artery.  The  patient,  a  man  aged 
thirty-two  years,  had  had  cyanosis  and  dyspnoea  from  birth.  There 
was  visible  precordial  pulsation  and  bulging,  and  a  systolic  murmur  over 
the  middle  of  the  heart  transmitted  everjn^v'here  over  its  surface,  but 
chiefly  to  the  right  and  inferiorly,  not  heard  above  its  base,  along  the 
course  of  the  aorta  nor  in  the  pulmonary  artery,  the  latter  vessel  being 
too  far  below  the  surface  to  transmit  the  sound. 

Aneurisms  of  the  Undefended  Space. — Cases  have  been  recorded 
by  Rokitansky,  Zahn,-  Hart,^  IMacCallum,*  and  others,  in  which,  in  the 
absence  of  any  evidence  of  endocarditis,  sacculations,  single  or  multi- 
locular,  of  the  membranous  portion  of  the  septum  project  into  the  right 
auricle  above  the  medial  cusp  of  the  tricuspid,  and  in  some  cases  extend 
also  into  the  musculature  of  the  interventricular  septum.  Malignant 
endocarditis  of  the  bulging  area  is  not  infrequent  and  rupture  into  the 
right  auricle  may  occur.  On  this  ground  it  has  been  argued^  that  these 
aneurisms  are  not  of  congenital  origin,  but  are  due  to  the  action  of  the 
inflammatory  process  upon  this  delicate  part  of  the  septum.  As  was 
pointed  out  by  Rokitansky,  however,  the  reverse  is  probably  true,  the 
aneurism  supplying  a  nidus  of  lowered  resistance  upon  which  an  infective 
process  has  been  secondarily  engrafted.  This  point  has  been  made  the 
subject  of  an  interesting  communication  by  ]\Iall,^  who  pronounces 
definitely  upon  the  congenital  and  non-inflammatory  origin  of  these 
aneurisms,  and  shows  the  cause  to  be  a  malposition  of  the  inferior  septum 
(inferior  septum  proper)  so  that  "the  membranous  septum  develops 
improperly  and  becomes  placed  in  a  horizontal  position,  and  is  thus 
weakened  in  every  way,  and  predisposed  to  the  formation  of  aneurisms." 
In  his  own  case  the  membranous  septum  was  cribriform,  and  the  hole 
communicated  with  numerous  sacs  in  the  medial  segment  of  the  tricuspid, 
and  also  bulged  into  the  right  atrium. 

Such  aneurisms  may  give  rise  to  marked  and  characteristic  physical 
signs.  This  was  true  of  two  remarkable  cases,  one  a  heart  which  is 
in  the  McGill  Museum,  the  other  reported  by  Tate,''  in  which  a  trumpet- 
shaped  tube,  which  formed  the  apex  of  a  saccular  pouching  of  the  pars 
membranacea,  projected  behind  and  perforated  the  medial  cusp  of  the 
tricuspid  valve ;  in  the  McGill  specimen  there  was  malignant  endocarditis 
of  the  immediately  adjacent  tricuspid  segment. 

1  Zeitschr.  f.  klin.  Med.,  1897,  xxxii,  Supp.  Heft,  1. 

2  Virchows  Arch.,  1878,  Ixxii,  206.  »  j^j^Z.,  1905,  clxxxi,  51. 

*  Johns  Hopkins  Hosp.  Bull,  1900,  xi,  69.  6  B^hl,  Zeits.f.  Biol,  1880,  xvi. 

6  Anat.  Record,  June,  1912,  p.  2921. 

7  Trans.  Path.  Soc.  London,  1892,  xliii,  36. 

VOL.  IV — 24 


370  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

The  relative  frequency  of  aneurisms  of  the  right  aortic  sinus  of  Valsalva 
and  the  probable  dependence  of  these  upon  the  juxtaposition  of  the  pars 
membranacea  of  the  right  aortic  cusp,  has  been  discussed  by  Hart.^ 
The  insertion  of  the  medial  cusp  of  the  tricuspid  just  behind  this  point 
may  further  weaken  this  region  by  making  it  a  seat  of  traction.  It  is 
not  necessary  to  presuppose  a  congenital  defect,  although  this  may  occur. 
In  a  case  reported  by  Hale  White^  an  oval  defect  admitting  a  No.  10 
catheter  lay  just  below  the  right  half  of  the  anterior  aortic  cusp:  its 
edges  were  thickened,  but  the  septum  around  it  for  three-quarters  of  an 
inch  was  thin  and  translucent,  and  the  sinus  of  Valsalva  above  it  was 
expanded  into  an  aneurismal  pouch,  which  protruded  and  burst  into  the 
right  ventricle.  The  author  thought  that  the  whole  septum  between  the 
lower  part  of  the  aorta  and  the  base  of  the  ventricles  was  abnormally 
thin. 

Defects  in  the  Septum  Elsewhere  than  at  the  Base. — These  are  usually 
multiple,  and  irregular  or  slit-like  in  form.  They  are  of  the  greatest 
rarity.    This  is  especially  true  of  those  low  down  in  the  septum. 

COMPLETE  ABSENCE  OR  RUDIMENTARY  DEVELOPMENT  OF   THE 

CARDIAC  SEPTA. 

A  rudimentary  development  of  the  cardiac  septa,  leading  to  a  diminu- 
tion in  the  number  of  the  heart's  cavities,  should  not  be  treated  entirely 
apart  from  localized  septal  defects,  being  simply  a  more  extreme  degree 
of  the  same  lesion.  Yet  the  cases  may  be  conveniently  grouped  together 
as  indicating  arrest  at  a  very  early  stage  of  embryonic  life  (fourth  week), 
frequently  associated  with  anomalies  elsewhere,  and  as  forming  an  alto- 
gether different  and  more  serious  picture. 

Cor  Biloculare. — Early  cases  were  recorded  by  Wilson  in  1798,  Farre 
in  1814,  Ramsbotham  in  1846,  and  Forster  in  1847,  and  there  are  six  in 
the  recent  literature  by  Rudolf,^  Ivonstantinowitsch,^  Gierke,^  Schroeder,^ 
Jensen,''  and  Rivet  and  Girard.^  We  have  had  an  opportunity  of  examin- 
ing the  specimen  recorded  by  Rudolf,  which  is  in  the  Museum  of  the 
Toronto  University.  The  patient  was  a  girl  of  sixteen  years,  undeveloped 
and  cyanotic,  who  suffered  from  marked  dyspnoea  and  died  of  pulmonary 
tuberculosis.  The  auricular  septum  was  absent  and  a  large  right  was 
divided  from  a  smaller  left  auricular  portion,  with  corresponding  auricular 
appendages,  by  a  slight  ridge  on  the  posterior  wall.  A  single  large 
bicuspid  auriculoventricular  orifice  opened  into  the  left  side  of  a  single 
ventricle  which  gave  off  a  large  aorta  and  a  smaller  artery  from  its  right 
side,  in  transposed  relations,  and  separated  from  the  left  part  of  the  cavity 
by  a  shallow  muscular  ridge,  which  lay  in  the  posterior  wall  and  marked 
the  site  of  the  absent  interauricular  septum.  The  pulmonary  orifice 
was  stenosed.     The    cases    by    Gierke    and    Konstantinowitsch   were 

1  Virchows  Arch.,  1905,  clxxxii,  168. 

2  Trans.  Path.  Soc.  London,  1892,  xliii,  34. 

3  Anat.  Soc.  of  Gt.  Brit,  and  Ire.,  Nov.,  1899. 

'  Prag.  Med.  Woch.,  1906,  p.  657.  ^  Charite-Annalen,  1908,  xxxii,  229. 

6  Deut.  Arch.  J.  klin.  Med.,  1911,  ccv,  122  ">  Giessen  Thesis,  1912. 

8  Arch,  des  Mai.  du  Coeur.,  November  11,  1913. 


CONGENITAL  CARDIAC  DISEASE 


371 


identical  with  this,  except  that  the  great  vessels  were  not  transposed 
and  in  the  former  the  pulmonary  artery  was  atresic,  in  the  latter  the 
aorta.  In  other  cases  the  development  of  the  aortic  septum  was  com- 
pletely arrested  and  a  common  arterial  trunk  replaced  the  two  great 
vessels. 

An  anomalous  entrance  of  the  pulmonary  veins  into  the  superior  cava, 
innominate,  hepatic,  or  other  veins,  instead  of  into  the  left  auricle,  was 
noted  in  Wilson's  case,  and  in  most  of  those  recently  reported.  In 
that  by  Rivet  and  Girard,  of  a  cyanotic  infant  aged  twenty-five  days 
with  polycythemia,  these  veins  formed  a  common  trunk  which  ended 
in  the  lobus  spigelii  of  the  liver,  and  anastomosed  here  with  the  vense  cavse 
and  portse.  It  seems  possible  that  this  anomaly  may  be  the  primary 
one,  at  least  as  regards  the  non-development  of  the  auricular  septum,  for 
comparative  anatomy  shows  that  differentiation  of  the  auricles  is  evolved 
during  the  formation  of  a  pulmonary  circulation. 

Fig.  29 


"Incomplete  double  heart,"  showing  (A)  the  interventricular  septum,  defective  in  its  upper  half; 
B,  a  large,  thick-walled  aorta,  arising  from  both  ventricles  above  the  defect;  C,  a  single  auriculoventri- 
cular  cusp  arising  from  both  ventricles;  D,  stenosis  of  the  conus  of  the  pulmonary  artery;  R.A.,  the 
enlarged  right  auricle.  The  right  auricle  and  left  ventricle  are  much  hypertrophied  and  dilated;  the 
left  auricle  and  sinus  of  the  right  ventricle  rudimentary.  The  interauricular  septum  is  defective  in  its 
lower^alf.     (From  a  specimen  in  the  McGill  Pathological  Museum,  presented  by  Dr.  Andrewes.) 


Such  cases  of  pure  biloculate  heart  are  extremely  rare.  A  more 
common  form  is  that  in  which  the  septa  are  partly  developed,  and  an 
incomplete  division  has  occurred  into  four  cavities,  the  organ  still  remain- 
ing two-chambered  in  the  exercise  of  its  function  (incomplete  double 
heart).  A  good  example  is  shown  in  a  specimen  in  the  McGill  Museum, 
presented  by  Dr.  F.  W.  Andrewes  (Fig.  29).  Here  the  auricles  are  incom- 
pletely divided  into  a  large  right  and  a  small  left  chamber,  by  a  narrow 
septum  having  a  large  defect  above,  multiple  fenestrations,  and  a  deeply, 
concave  lower  free  border  (persistent  ostium  primum) .  A  thick  muscular 


372  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

septum,  one  inch  high,  with  rounded  free  border,  projects  upward  from 
the  lower  wall  of  the  ventricle,  partly  dividing  it  into  a  small,  thick-walled 
right,  and  a  capacious  left  ventricle.  A  dilated  aorta  rides  above  this 
rudimentary  septum  and  a  narrow  thin-walled  bicuspid  pulmonary  arises 
from  a  rudimentary  conus.  There  is  a  common  auriculo ventricular 
orifice  with  five  cusps,  one  of  which  is  very  strong  and  large  and  arises 
from  the  opposing  wall  of  either  ventricle,  stretching  across  above  the 
rudimentary  septum  and  shielding  the  auriculoventricular  orifice  from 
the  two  arterial  ostia. 

A  third  variation  of  biloculate  or  triloculate  heart  is  presented  by 
cases  of  mitral  or  tricuspid  atresia  with  absent  or  defective  auricular 
septum.  In  tricuspid  atresia  the  ventricular  septum  has  been  developed, 
but  a  defect  remains  at  the  base  through  which  the  blood  passes  from  the 
large  left  ventricle  into  the  pulmonary  artery  through  the  persistent 
bulbus,  the  sinus  of  the  right  ventricle  having  become  obliterated.  An 
excellent  example  of  the  former  condition  (tricuspid  atresia)  is  published 
by  Robertson^  under  the  title,  cor  biatriatrum  triloculare.  The  auricular 
septum  was  defective  below  (persistent  ostium  primum)  and  an  anomalous 
septum,  evidently  the  persistent  right  valvula  venosa,  crossed  the  right 
auricle  from  the  Eustachian  valve,  and  was  inserted  into  the  base  of  the 
interauricular  septum  at  the  site  of  the  tricuspid  orifice,  which  was  here 
obliterated,  possibly  as  a  result  of  the  insertion  of  the  afiomalous  septum 
at  this  point.  The  great  arterial  trunks  were  transposed,  the  aorta 
arising  from  the  persistent  bulbus  arteriosus  of  the  obliterated  right 
ventricle,  which  communicated  with  the  cavity  of  the  left  ventricle  by  a 
defect  at  the  base  of  the  otherwise  fully  developed  interventricular 
septum.  Aplasia  of  the  left  chambers  in  mitral  atresia  is  described  by 
Bernstein^  and  by  Girauld  and  Tissier.^  In  the  latter's  case  the  left 
auricle  was  tiny,  and  was  separated  from  the  left  ventricle  by  a  rudimen- 
tary bicuspid  valve,  and  a  large  orifice  united  it  with  the  greatly  dilated 
right  auricle.  The  right  ventricle  was  also  enormous  and  gave  off  both 
the  aorta  and  the  pulmonary  artery  in  normal  relations  but  separated 
from  each  other  by  a  thick  muscular  cushion  (apparently  marking  the 
conus  of  the  pulmonary  artery). 

Biloculate  heart  is  frequently  displaced  to  the  right  side  of  the  body, 
in  that  type  of  dextrocardia  that  is  apparently  due  to  arrest  of  develop- 
ment. It  is  often  associated  with  transposition  of  one  or  more  viscera 
or  other  serious  defects.     The  spleen  was  absent  in  three  of  the  cases. 

Cor  Biatriatrum  Triloculare. — Absence  of  the  ventricular  with  presence 
of  the  auricular  septum  constitutes  a  three-chambered  heart  with  two 
auricles  and  the  tricuspid  and  mitral  orifices  opening  into  a  common 
ventricle,  from  which  (if  the  aortic  septum  develops),  two  arterial  trunks 
arise.  Arnold^  reports  a  case  of  complete  absence  of  the  ventricular 
septum,  with  auricular  septum  defective  below  (persistent  ostium 
primum),  pulmonary  atresia,  dextrocardia,  and  absence  of  the  spleen, 
and  adds  a  study  of  30  case^  of  cor  biatriatrum  triloculare.     As  in  cor 

^Lancet,  1911,  180,  p.  872. 

2  Proc.  Neiv  York  Path.  Soc,  1906,  N.  S.,  vi,  p.  29. 

3  Bull.  Soc.  d'Obstet.  de  Paris,  1910,  xiii,  420.  ^  Virchows  Arch.,  xlii. 


CONGENITAL  CARDIAC  DISEASE 


373 


biloculare,  obliteration  of  the  right  ventricle  in  tricuspid  atresia  (with 
auricular  septum  present)  is  frequently  reported  as  triloculate  heart. 

A  special  type  of  anomaly  has  been  established,  of  what  is  functionally 
a  cor  biatriatrum  triloculare,  although  four  chambers  exist,  by  a  series 
of  cases  recorded  in  which  an  anomalous  septum  cuts  off  from  a  common 
ventricle  a  small  chamber  which  lies  at  the  base  of  the  heart  and  gives 
off  one  or  the  other  of  the  great  arterial  trunks.  Such  a  case,  reported 
and  figured  by  Holmes,^  in  1824,  the  specimen  from  which  is  in  the  McGill 

Fig.  30 


Cor  biatriatrum  triloculare  with  malposed  interventricular  septum.  Diagrammatic  sketch  by  Prof. 
R.  Tait  Mackenzie,  showing  course  of  blood  and  relation  of  cavities  in  Dr.  Andrew  Holmes'  case  of 
displaced  interventricular  septum  cutting  off  a  small  cavity  which  gives  off  the  pulmonary  artery.  The 
pale  line  shows  venous,  the  dark  line  arterial  blood.     (From  the  Medical  Museum  of  iMcGill  University.) 

Museum,  is  represented  diagrammatically  in  Fig.  30.  The  auricles, 
which  were  enormously  dilated,  especially  the  right,  emptied  their  con- 
tents through  their  respective  ostia  into  a  common  ventricle,  which  gave 
off  the  aorta  behind  and  somewhat  to  the  left  and  communicated  through 
a  diamond  shaped  opening  in  an  anomalous  septum  with  a  small  cavity 
at  its  right  upper  angle  of  the  common  ventricle  which  gave  off  the 
dilated  pulmonary  artery  in  its  normal  relation  to  the  aorta.     In  eight 

^  Edin.  Med.  Chir.  Soc,  1824,  republished  by  M.  E.  Abbott,  Montreal  Med.  Jour., 
1901. 


374  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

other  similar  cases  reported  by  Young/  Peacock,^  Rokitansky  (2  cases), 
Chiari,  Theremin  (Obs.  43),  and  ]Marchand,^  and  in  a  specimen  which 
the  writer  has  had  the  privilege  of  studying  in  the  Museum  of  the  Har- 
vard Medical  School,  the  aorta  arose  from  the  small  chamber  in  front 
and  to  the  right,  and  the  pulmonary  artery  from  the  common  ventricle 
behind  it,  the  two  vessels  being  in  transposed  relations.  It  has  been 
suggested  by  Keith  that  in  these  cases  the  anomalous  septum  is  the 
persistent  lower  orifice  of  the  embryonic  bulbus  cordis,  but  study  of  both 
the  McGill  and  Harvard  specimens  lead  us  to  the  conclusion,  that  in  these 
specimens,  at  least,  the  strong  muscular  wall  with  a  large  defect  at  its 
upper  border  through  which  the  small  cavity  communicates  with  the 
large  common  ventricle,  is  simply  the  malposed  interventricular  septum 
itself,  which  has  failed  to  unite  with  the  aortic  septum,  and  has  been 
carried  around  to  the  right  in  the  further  development  of  the  heart. 

Cor  Biventriculare  Triloculare. — When  the  auricular  septum  is  absent 
and  the  ventricular  septum  present,  a  heart  with  two  ventricles  and  a 
single  auricle  results.  Such  a  case  is  reported  by  Williams.'^  All  the 
blood  entered  the  common  auricle  through  the  superior  vena  cava  (the 
pulmonary  veins  having  again  an  anomalous  entrance). 

Symptoms  and  Physical  Signs. — In  biloculate  heart  cyanosis  is  usually 
present  from  birth,  and  becomes  very  marked.  The  cases  of  Forster,^ 
Ramsbotham,^  and  Crisp^  were  all  typical  morbus  cseruleus,  dying, 
respectively,  at  seventy-eight  hours,  ten  days  and  ten  weeks.  On  the 
other  hand  symptoms  may  be  moderate,  as  in  Turner's  case,  in  which 
there  was  no  cyanosis  until  just  before  death  at  the  age  of  fifteen  months. 
Cases  of  cor  biatriatrum  triloculare  present  perhaps  the  best  illustrations 
we  have  that  the  adniixture  of  venous  and  arterial  blood  is  compatible 
with  long  life  and  with  only  slight  disturbances  of  the  circulation.  Young's 
patient,  who  died  at  thirty-nine  years,  cyanosis  having  developed  only 
within  the  last  three  weeks  of  life,  and  Peacock's  almost  identical  case, 
already  noted,  and  that  by  Mann,^  dying  at  twenty-one  years,  are  illus- 
trations. Holmes'  specimen  (Fig.  30)  was  from  a  young  man,  aged 
twenty-four  years,  in  whom  there  was  only  moderate  cyanosis  and  a 
tendency  to  suffocative  attacks.  Physical  signs  may  be  prominent, 
but  cannot  be  said  to  be  characteristic. 

DEFECTS  OF  THE  AORTIC  SEPTUM. 

When  the  aortic  septum  fails  to  develop,  a  single  large  thick-walled 
trunk  {persistent  truncus  arteriosus)  results,  which  arches  upward  in  the 
course  and  gives  off  the  branches  of  the  normal  aorta,  the  pulmonary 
artery  arising  therefrom.  This  abnormality  is  very  uncommon.  Partial 
defect  of  the  aortic  septum  is  even  less  frequent  than  is  its  complete 
absence.     It  may  result  in  a  common  trunk  with  early  division  into  aorta 

1  Jour.  Anat.  and  Physiol.,  1907,  xli,  190. 

2  Trans.  Path.  Soc,  London,  1854,  vi,  177. 

3  Verh.  d.  XII  Deut.  path.  Gesell,  1908,  p.  174. 
*  Jour.  Anat.  and  Physiol.,  1894,  xxviii,  305. 

•    5  Trans.  Path.  Soc,  1846,  i,  21.  s  Ihid.,  p.  21. 

'  Trans.  Path.  Soc,  London,  1859,  x,  49.  «  Ziegler's  Beitr.,  1889,  vi,  485. 


COMGUNiTAL  CARDIAC  DISEASE 


375 


and  pulmonary  artery  and  rudimentary  septum  within  it,  or,  when  the 
defect  is  still  smaller,  in  an  abnormal  communication  between  the  aorta 
and  the  pulmonary  artery  or  the  conus  of  the  right  ventricle. 

Persistent  Truncus  Arteriosus  (Common  Arterial  Trunk,  Complete 
Defect  of  the  Aortic  Septum). — This  abnormality  is  very  uncommon, 
only  22  cases  are  available  in  the  literature  and  from  other  sources 
of  study.  The  cardiac  septa  are  frequently  rudimentary  or  absent, 
a  biloculate  or  triloculate  heart  existing.  When,  however,  these  are 
well  developed  and  the  heart  is  four-chambered  and  otherwise  normal, 
a  localized  defect  at  the  base  of  the  interventricular  septum  always 
remains.  The  common  trunk  either  rides  above  it,  receiving  the  blood 
equally  from  both  ventricles,  or  arises  more  or  less  entirely  from  the 
right  ventricle,  the  blood  entering  it  from  the  left  through  the  defect. 


Fig.  31 


Persistent  truncus  arteriosus.  Heart  of  a  ciiild,  aged  five  years,  in  whom  cyanosis  developed  at  one 
and  one-half  years.  A,  common  arterial  trunk  arising  entirely  from  right  ventricle,  and  communicating 
with  the  left  ventricle  through  the  right  auricle;  B,  right  auricle  laid  open;  C,  defect  at  base  of  inter- 
ventricular septum;  D,  heavy  muscular  column  from  wall  of  right  ventricle  to  base  of  defect;  E,  inter- 
auricular  septum  seen  from  right  auricle  showing  multiple  defects.  Heart  is  wider  than  high.  (From  a 
specimen  in  the  McGiU  Pathological  Museum  presented  by  Dr.  Mackenzie  Forbes.) 


This  recalls  the  early  stage  at  which  the  arrest  of  growth  has  occurred, 
when  the  primitive  aorta  is  given  off  entirely  from  the  right  side  of  the 
common  ventricle  (Fig.  16).  A  typical  specimen  of  this  kind  is  shown 
in  Fig.  31  from  a  specimen  in  the  McGill  Museum.  The  large  common 
arterial  trunk  springs  entirely  from  the  right  ventricle,  and  has  three 
strong  semilunar  cusps,  behind  two  of  which  the  coronaries  arise.  Below 
the  anterior  and  right  posterior  cusps  there  lies  a  circular  defect  in  the 
septum,  admitting  the  little  finger,  with  rounded  edges,  by  which  the 
left  ventricle  communicated  with  the  aorta  and  with  the  right  ventricle, 
which  is  much  hypertrophied;  a  heavy  muscular  column  runs  from  the 
anterior  wall  of  this  ventricle  to  the  lower  border  of  the  defect.  The 
left  auricle  and  ventricle  are  perfectly  formed,  but  are  much  smaller 
than  the  right  chambers.  There  are  multiple  defects  in  the  inter  auricular 
septum.     The  blood  supply  to  the  lungs  is  unknown. 


376  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

The  pulmonary  circulation  in  these  cases  is  of  the  greatest  interest. 
Two  large  branches,  one  to  either  lung,  may  arise  from  the  wall  of  the 
common  trunk  some  distance  above  its  origin,  as  in  the  cases  of  Heath^ 
and  Ramsbotham.  Quite  commonly  one  pulmonary  branch  arises  from 
the  aorta  soon  after  its  origin,  and  supplies  either  the  right  or  the  left 
lung,  and  a  second  branch  passes  to  the  lung  of  the  opposite  side  from 
the  descending  aorta,  the  first  vessel  evidently  representing  the  pulmonary 
portion  of  the  truncus  (sixth  aortic  arch)  and  the  second  the  patent 
arterial  duct.  This  occurred  in  Dickson's^  and  Wirth's^  cases,  and  also 
in  that  by  Preisz.*  In  the  latter,  distinct  traces  of  a  partial  development 
of  the  aortic  septum  were  apparent.  A  common  trunk  provided  with 
four  semilunar  cusps,  14  mm.  in  diameter,  arose  from  the  right  ventricle 
above  a  large  septal  defect,  and  widened  rapidly  to  3  cm.,  becoming 
grooved  externally  to  indicate  the  right  and  left  portions  of  its  lumen, 
that  part  on  the  right  giving  off  the  vessels  of  the  arch,  and  thus  repre- 
senting the  aorta,  while  that  on  the  left  yielded  the  vessels  to  the  right 
lung  and  represented  the  pulmonary  artery.  An  additional  pulmonary 
artery  was  here  also  sent  to  the  lungs  from  the  descending  aorta  in  the 
position  of  the  ductus  arteriosus. 

In  a  few  instances  in  which  the  defect  in  the  aortic  septum  appears 
to  be  only  partial,  the  common  trunk  divides  immediately  after  its  origin 
from  the  heart,  two-thirds  of  its  lumen  on  the  right  forming  the  aorta, 
and  one-third  on  the  left  forming  the  pulmonary  artery,  which  takes 
its  normal  course. 

This  early  division  occurs  in  both  of  Rokitansky's  cases,  and  in  them 
the  partial  character  of  the  defect  is  also  proved  by  the  presence  of  a  deli- 
cate sickle-shaped  septum  within  the  undivided  portion  of  the  trunk, 
joining  its  wall  between  the  left  and  posterior  semilunar  cusps  behind, 
and  left  and  right  cusps  in  front.  In  Clarke's^  case  the  trunk  divided 
early,  but  there  was  no  sign  of  a  rudimentary  septum  within. 

The  above  are  examples  of  true  persistent  truncus  arteriosus.  Under 
this  title  is  also  reported  a  series  of  cases  in  which  the  single  trunk  passing 
from  the  heart,  represents  the  greatly  dilated  aorta  or  pulmonary  artery, 
as  the  case  may  be,  and  an  atresic  cord  attached  to  the  external  surface 
of  the  heart  represents  the  obliterated  origin  of  the  other  vessel.  In  one 
of  Farre's  cases,  for  instance,  and  in  one  by  Forster,  the  single  large 
trunk  represented  the  pulmonary  artery,  and  "a  single  coronary  vessel 
which  descended  from  the  concavity  of  the  arch  to  the  base  of  the  heart 
where  it  divided  into  two  coronary  arteries"  was  evidently  the  aorta, 
which  was  atresic  at  its  origin,  and  was  connected  with  the  pulmonary 
by  a  large  patent  ductus.  In  Crisp's  case,  on  the  other  hand,  the  pulmo- 
nary artery  was  rudimentary,  and  the  large  trunk  represented  the  aorta. 

As  pointed  out  by  Gierke,  and  also  by  Wirth,  such  cases  should  be 
clearly  distinguished  from  the  true  persistent  truncus  due  to  complete 
absence  of  the  aortic  septum.     Where  the  interventricular  septum  is 

1  Trans.  Path.  Soc,  London,  1864,  xii,  62. 

2  Jour.  Anat.  and  Physiol.,  1914,  xlviii,  p.  210. 

3  Giessen  Thesis,  1912.  ^  Ziegler's  Beitrage,  1890,  vii,  247. 
^  Trans.  Path.  Soc,  London,  1885,  xxvi,  178. 


CONGENITAL  CARDIAC  DISEASE  377 

defective  at  its  base,  as  it  is  in  all  these  cases,  there  is  a  tendency  for  the 
aortic  septum  to  develop  irregularly,  thus  cutting  off  a  narrow  aorta 
or  pulmonary  artery,  as  the  case  may  be,  and  the  calibre  of  the  smaller 
vessel  is  likely  to  become  still  further  reduced  in  size  by  the  passage  of 
the  bulk  of  the  circulation  into  the  larger  trunk.  For  this  reason  oblitera- 
tion of  one  trunk  in  biloculate  heart  where  no  interventricular  septum 
is  present,  is  a  comparatively  common  event,  and  the  cases  should  be 
sharply  distinguished  from  a  true  defect  of  the  aortic  septum.  Gierke 
suggests  that  the  presence  of  four  semilunar  cusps  such  as  occurred  in 
Preisz's  case  is  positive  proof  of  an  undivided  primitive  arterial  trunk, 
while  the  presence  of  three  semilunar  cusps  such  as  occurred  in  the  McGill 
specimen,  his  own,  Dickon's,  and  Wirth's  argues  that  development  of 
the  aortic  septum  had  occurred  and  that  obliteration  of  one  or  other  of 
the  vessels  had  taken  place  as  a  secondary  event. 

Symptoms  and  Physical  Signs. — Much  the  same  remarks  apply  to 
persistent  truncus  as  to  bilocular  heart,  for  sjTuptoms  and  signs  are  not 
always  commensurate  with  the  seriousness  of  the  lesion.  But  here  the 
condition  is  a  still  graver  one  and  the  average  duration  of  life  is  much 
shorter.  Crisp's  patient,  a  girl  dumb  from  birth,  with  only  slight  cyanosis 
and  clubbing,  lived  to  twelve  years,  Forbes'  to  five  years,  Peacock's 
to  thirteen  months,  and  Buchanan's,  who  had  a  four-chambered  heart  with 
defect  at  the  base  of  the  septum,  giving  ph}'sical  signs  but  no  cyanosis, 
to  six  and  a  half  months;  all  the  others  were  marked  cases  of  morbus 
coeruleus  and  died  at  birth  or  in  early  infancy.  Vierordt  quoted  one 
dying  at  sixteen  and  another  at  nineteen  years. 

Communication  between  the  Aorta  and  Pulmonary  Artery. — (Partial 
Defect  of  the  Aortic  Septum. — A.  few  cases  have  been  described  in  which 
a  circular  or  oval  hole  with  perfectly  smooth  edges,  and  evidently  not 
of  inflammatory  origin  lies  in  the  anterior  wall  of  the  aorta  a  short  distance 
above  the  semilunar  cusps,  and  leads  directly  into  the  pulmonary  artery 
shortly  above  its  origin.  A.  valuable  developmental  study  was  made  by 
Hektoen,^  who  gives  a  case  of  much  interest  and  collected  9  others  from 
the  literature. 

The  effect  produced  upon  the  circulation  by  the  abnormal  communica- 
tion between  the  two  great  vessels  is  the  same  as  in  a  patent  ductus 
arteriosus,  but  the  two  conditions  are  quite  distinct  and  have  an  entirely 
different  etiology.  This  lesion  is  not  a  patent  ductus  abnormally  shOTt- 
ened  so  that  aorta  and  pulmonary  artery  have  been  approximated,  with 
an  apparent  hole  as  a  result.  It  is  a  true  defect,  as  is  proved  both  by 
its  situation,  which  is  much  nearer  to  the  origin  of  both  arteries  than  is 
the  ductus,  and  by  the  fact  that  in  several  of  the  cases  reported  the  ductus 
has  been  present  as  well.  Neither  is  the  defect  of  an  inflammatory 
nature,  as  is  well  seen  in  the  smooth  condition  of  its  edges  in  the  cases  of 
Girard,  Wilks,  and  Rickards,  and  from  the  character  of  the  combined 
defects  in  the  latter  and  in  Fraentzel's  case. 

The  abnormal  opening  is  clearly  a  partial  defect  of  the  aortic  septum, 
not  at  its  junction  with  the  interventricular  septum,  but  higher  up  in 

1  Trans.  Chicago  Path.  Soc,  1905. 


378  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

its  own  substance,  probably  at  the  point  where  the  distal  bulbar  swellings 
meet  the  aortic  septum  proper  in  the  embryo. 

In  all  the  recorded  cases  but  one,  the  hole  lay  in  the  aortic  wall  a 
short  distance  above  the  semilunar  cusps.  In  that  of  Richards  the 
aortic  valve  was  bicuspid,  and  its  segments  congenitally  fused.  A  funnel- 
shaped  opening  communicated  with  the  pulmonary  artery  and  lay  behind 
the  larger  (fused)  cusp.  A  triangular  defect  in  the  interventricular  septum 
existed  also  in  this  case. 

Instead  of  opening  into  the  pulmonary  artery,  the  hole  may  lead  from 
the  aorta  into  the  right  ventricle  as  in  the  cases  of  Livingstone^  and 
Cayla.  In  one  of  Hektoen's  cases  the  anomalous  opening  led  from  the 
aorta  into  the  right  ventricle  through  a  defect  at  the  base  of  one  of  the 
semilunar  valves.  This,  also,  is  a  most  interesting  example  of  a  defect 
of  the  lower  (bulbar)  part  of  the  aortic  septum. 

A  communication  between  the  aorta  and  pulmonary  artery  of  an 
acquired  nature  may  occur  in  much  the  same  situation  as  in  the  congenital 
form  in  aneurism  of  the  base  of  the  aorta.  There  is  quite  a  large  series 
of  cases,  especially  in  the  earlier  literature,  which  is  reviewed  by  Brocq.^ 
The  congenital  cases  are  to  be  distinguished  from  those  due  to  perforation 
of  an  aneurismal  sac  by  the  smooth  appearance  of  the  edges  of  the  opening 
and  the  healthy  arterial  wall.  The  physical  signs  produced  are  the  same 
and  are  often  of  remarkable  intensity.  Gairdner^  reported  a  typical 
case,  characterized  by  a  continuous  murmur. 

The  pulmonary  artery  was  larger  than  the  aorta  in  Wilks'  and  Richard's 
cases,  a  little  smaller  in  Fraentzel's  and  Girard's  cases.  Marked  hyper- 
trophy and  dilatation  of  all  the  chambers  of  the  heart,  especially  of  the 
right  ventricle,  constantly  results.  In  Girard's  and  Richards'  cases, 
.  in  which  there  was  no  other  cardiac  lesion,  the  hearts  weighed,  respec- 
tively, 670  gms.  and  23  oz.  (651.8  gm.). 

Symptoms  and  Physical  Signs. — Cyanosis  is  not,  as  a  rule,  present,  and 
there  is  no  characteristic  picture,  excepting  that  obstruction  to  the  circu- 
lation is  clearly  manifest.  All  the  cases  recorded  died  before  middle 
life  with  anasarca  and  other  sjTiiptoms  of  chronic  heart  disease;  those 
of  Girard,  Richards,  and  Fraentzel  reached  thirty-seven,  thirty,  and 
twenty-five  years  respectively.  Wilks'  infant  died  at  eight  months, 
Hektoen's  and  Lebederber's  at  birth.  Precordial  discomfort,  amounting 
often  to  actual  distress,  was  present  since  childhood  in  Richards'  patient, 
as  also  in  Fraentzel's  and  Girard's,  and  in  the  two  latter  there  was  dyspnoea 
on  exertion  and  slight  cyanosis.  Physical  signs  may  be  absent  or  may 
be  very  marked,  and  they  may  vary,  being  sometimes  quite  atypical, 
or  sometimes  those  usually  associated  with  a  patent  ductus  arteriosus. 
Owing  to  the  great  hypertrophy  of  the  heart  the  cardiac  dulness  is  usually 
much  increased,  especially  to  the  right,  and  there  is  precordial  bulging. 
Girard's  case  was  characterized  by  a  slight  thrill  and  systolic  murmur 
at  the  apex,  which  latter  gave  place  later  to  reduplication"  of  the  first 
sound  and  distinct  gallop  rhythm. 

1  New  York  Med.  Rec,  1883,  p.  249. 

2  Reuue  de  med.,  1885,  v,  1046  and  1886;  vi,  786. 
^  Glasgow  Hospital  Reports,  1899. 


CONGENITAL  CARDIAC  DISEASE  379 

A  diastolic  murmur  is  common.  In  Rickards'  case  the  cardiac  dulness 
was  enormously  increased,  and  there  was  an  intense  purring  double 
thrill  over  the  cardia,  and  mm-murs,  systolic  and  diastolic,  running  into 
each  other,  were  heard  over  the  whole  front  and  back  of  the  chest,  so  loud 
as  to  be  audible  even  through  the  bedclothes.  In  Fraentzel's  patient, 
in  whom  there  was  a  gaping  opening  12  mm.  across  between  the  two 
vessels,  and  the  right  pulmonary  artery  arose  from  the  ascending  aorta, 
the  heart  was  found  to  be  greatly  enlarged;  both  sounds  were  heard  at 
all  the  ostia,  and  a  systolic  murmur  was  heard  at  the  apex;  in  the  fourth 
left  space  near  the  sternal  border  was  a  loud  systolic  and  a  long  diastolic 
murmur,  the  latter  heard  with  equal  intensity  over  the  base  of  the  xiphoid 
cartilage.  Both  sounds  and  a  diastolic  murmur  were  audible  in  both 
second  spaces,  the  mm-mur  being  louder  and  rougher  in  the  second  right 
space  than  in  the  left.  Both  sounds  as  well  as  systolic  and  diastolic 
murmurs  were  audible  in  the  carotids. 

DEVIATION  OF  THE  AORTIC  SEPTUM. 

According  to  the  teaching  of  Rokitansky,  transposition  of  the  arterial 
trunks,  Rechtslage  or  deviation  to  the  right  of  the  aorta,  as  well  as  certain 
developmental  forms  of  pulmonary  and  aortic  stenosis,  are  due  to  irregu- 
larities in  the  development  of  the  septum  within  the  aortic  bulb,  or  to 
its  malunion  with  the  interventricular  septum.  As  this  theory  with 
certain  modifications  still  finds  general  acceptance,  the  cases  embraced 
by  it  will  be  considered  here. 

Transposition  of  the  Arterial  Trunks. — This  may  be  defined  as  an 
alteration  in  the  position  of  the  two  great  vessels  relative  to  the  ventricles 
of  the  heart  or  to  each  other  at  their  origin,  so  that  they  either  spring 
from  reversed  ventricles,  the  aorta  from  the  right  and  the  pulmonary 
from  the  left  chamber  (complete  transposition),  or  from  the  ventricle 
to  which  they  normally  belong,  but  in  a  reversed  relationship  ("  corrected" 
transposition) . 

The  condition  was  first  described  by  Baillie  in  1797,  and  early  cases  were 
reported  by  Farre  in  1814,  Ward^  in  1851,  Peacock^  in  18.54,  Buchanan^ 
and  Meyer^  in  1857,  Cockle^  in  1863,  and  Kelle/  in  1870.  As  a 
result  of  systematic  observation  of  the  autopsy  material  of  infants  at  the 
St.  Petersburg  Foundling  Hospital,  Theremin  found  26  cases  of  trans- 
position among  106  cardiac  defects.  In  his  summary  of  the  literature 
in  1898,  Vierordt  mentions  (p.  47)  76  cases  of  transposition  among  383 
cardiac  defects  analyzed.  Some  thirty  additional  cases  have  been 
reported  since.  Among  270  cardiac  defects  in  the  London  ]\Iuseimis 
examined  by  Keith,  transposition  occm-red  in  twenty-five.  In  our 
series  of  defects  it  was  present  seventy  times. 

Pathogenesis. — Peacock  and  others  ascribed  this  anomaly  to  an  irregu- 
larity in  the  development  of  the  aortic  septum,  but  until  Rokitansky's 

1  Trans.  Path.  Soc,  London,  1851,  v,  67. 

2  Ibid.,  1854,  vi,  117.  ^  jud.,  1857,  viii,  149. 

*  Virchows  Arch.,  1857,  xii,  364.  ^  Medico-Chir.  Trails.,  1863,  xlvi,  193. 

^  Trans.  Path.  Soc,  London,  1871,  xxii,  92. 


380  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

work  appeared  in  1875,  it  remained  a  little  understood  phenomenon. 
In  some  minor  particulars  the  observations  of  Rokitansky  upon  develop- 
ment do  not  coincide  with  those  of  later  observers,  but  it  is  in  the  elucida- 
tion of  the  complex  and  hitherto  obscure  subject  of  transposition  of  the 
arterial  trunks  that  the  value  of  his  great  achievement  may  be  said 
chiefly  to  lie.  Rokitansky's  explanation  of  transposition  is  one  of  those 
revelations,  astonishing  by  its  simplicity,  and,  as  Vierordt  remarks, 
he  had  the  singular  triumph  of  having  supplied  a  working  hypothesis 
that  has  not  only  explained  the  facts  of  his  own  experience,  but  has 
outlined  and  foreseen  other  pathological  possibilities  which  have  since 
been  realized.  He  described  and  figured  sixteen  different  forms  of 
transposition,  due,  he  believed,  to  different  degrees  and  combinations 
of  deviation  or  malunion  of  the  aortic  and  interventricular  septa,  some 
of  which  he  himself  observed,  and  others  have  since  been  recorded  by 
later  workers.  Even  if  the  advance  of  comparative  embryology  should 
unfold  some  other  explanation  of  this  subject,  the  verification  of  Roki- 
tansky's brilliant  hypothesis  by  the  subsequent  observation  of  different 
forms  of  transposition  showm  by  it  to  be  possible,  indicates  that,  so  far 
as  it  goes,  his  theory  is  true,  and  that  growing  knowledge  will  amplify 
rather  than  supersede  his  solution  of  this  difficult  problem. 

Since  the  above  statement  was  written  for  the  first  edition  of  this 
work,  a  remarkably  clear  exposition  of  Rokitansky's  theory  in  the  light 
of  recent  investigations  upon  the  normal  anatomy  of  the  bulbus  cordis 
in  the  dipnoan  and  reptilian  heart  and  in  the  mammalian  embryo,  has 
been  published  by  Jane  Robertson.^  The  following  is  a  brief  statement 
of  Rokitansky's  doctrine  of  transposition,  as  corroborated  and  amplified 
by  the  result  of  these  researches. 

As  may  be  seen  by  a  glance  at  the  normal  adult  heart,  the  great  trunks 
normally  undergo  a  distinct  torsion  upon  each  other  just  above  their 
origin,  so  that  the  pulmonary  artery,  w^hich  arises  in  front  and  to  the 
left,  passes  back  behind  the  ascending  aorta  to  the  lungs,  while  the  aorta, 
although  coming  to  lie  ventral  to  the  pulmonary  artery,  springs  from  the 
heart  behind  it  and  to  the  right.  This  torsion  of  the  vessels  is 
represented  in  the  bulbus  cordis  of  the  early  embryo  and  in  the  dipnoan 
fish  by  a  spiral  arrangement  of  the  valvular  endocardial  folds  of  the  bulbus 
cordis,  which  results  from  a  kinking  upon  itself  of  the  bulbus,  which  struct- 
ure was  at  an  earlier  stage  a  straight  tube.  The  aortic  septum  is  formed 
in  its  proximal  portion  by  fusion  of  the  spirally  placed  endocardial  ridges 
of  the  bulbus  cordis,  and  in  its  distal  part  by  a  growth  downward  of  a 
septum  in  the  common  arterial  trunk.  If  for  any  reason  this  normal 
kinking  does  not  occur  and  the  bulbus  remains  a  straight  tube,  the  aortic 
septum  will  not  assume  its  spiral  form,  and  the  normal  torsion  of  the 
great  trunks  upon  each  other  cannot  take  place.  The  result  is  an  aorta 
arising  anteriorly  and  passing  directly  upward  to  the  arch,  and  a  pul- 
monary artery  arising  posteriorly  and  passing  directly  backward  to  the 
lungs,  i.  e.,  transposition.  In  other  words,  transposition  of  the  arterial 
trunks  is  due  to  a  lack  of  development  of  the  torsion  that  normally 

^  Jour.  Path,  and  Bacleriol.,  1913,  xviii,  191. 


CONGENITAL  CARDIAC  DISEASE 


381 


occurs.  The  transposed  vessels  may  be  placed  in  their  proper  ventricles 
in  spite  of  their  relative  displacement,  by  the  sympathetic  adjustment 
of  the  aortic  in  its  union  with  the  interventricular  septum.  In  this  case 
the  transposition  is  "corrected"  (Rokitansky's  Scheme  A).  Or  the 
transposed  vessels  may  be  placed  in  the  reversed  ventricles  by  the  union 
of  the  interventricular  with  the  proximal  portion  of  the  malposed  aortic 
septum,  when  "uncorrected"  (anomalous)  transposition  results  (Roki- 
tansky's Scheme  B). 


Fig    32 


Fig.  33 


Normal  relation. 


Corrected  transposition 


Fig.  34 


True  complete  transposition,  vessels  in  reversed  ventricles. 
A,  aorta;  P,  pulmonary  artery;   T,   tricuspid  valve;    B,  bicuspid  valve;   pm,  pars   membranacea: 
p,  posterior  cusp;  a,  anterior  cusp;  Ir,  left,  right  cusps  (the  small  circles  indicate  the  situation  of  the 
coronary  article.)     (Republished  from  Vierordt,  Nothnagel's  System,  1898,  xv,  1,  2.) 


Rokitansky  recognized  this  spiral  disposition  of  the  aortic  septum 
and  taught  that  these  two  factors,  a  deviation  of  the  septum  within  the 
aortic  bulb  and  its  faulty  union  with  the  interventricular  septum,  might 
occur  in  all  degrees  and  combinations,  giving  rise  to  a  corresponding 
number  of  different  forms  of  displacement  of  the  arterial  trunks  or  of 
"corrections"  of  such  displacements.  He  distinguished  two  main  classes 
(according  as  the  transposition  is  "corrected"  or  otherwise  by  the  inter- 
ventricular septum),  with  eight  sub-varieties  in  each.  Of  these  his 
"Scheme  A"  has  as  its  type  or  starting  point  the  normal  relation,  in 
which  the  concavity  of  the  septum  looks  backward  and  to  the  right. 
The  different  subvarieties  are  constituted  by  the  different  degrees  of 


382  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

deviation  of  the  aortic  septum  rotating  in  an  imaginary  circle  from  right 
to  left.  The  characteristic  of  the  group  as  a  whole  is  that  the  interven- 
tricular unites  with  the  aortic  septum  in  such  a  way  that,  although  the 
trunks  are  altered  in  their  relation  to  each  other,  they  remain  placed  in 
their  respective  ventricles:  that  is  to  say,  the  transposition  is  "corrected." 

In  his  second  group,  or  "Schema  B,"  on  the  other  hand,  the  arteries 
arise  throughout  from  the  "reversed"  ventricles;  that  is  to  say,  the  trans- 
position is  "uncorrected"  in  the  union  of  the  interventricular  septum. 
The  type,  or  fundamental  form  from  which  the  series  starts,  is  the  so-called 
transpositio  vera,  in  which  the  septum  has  rotated  through  180  degrees, 
so  that  its  concavity  looks  downward  and  to  the  left,  and  the  arteries 
lie  in  reversed  cavities  in  the  exact  opposite  of  the  normal  position,  the 
aorta  to  the  left  and  anteriorly,  the  pulmonary  to  the  right  and  posteriorly 
(Fig.  34) .  Another  explanation  of  transposition  has  been  offered  recently 
by  Keith,  namely,  that  it  is  due  to  an  atrophy  of  the  bulbus  cordis  around 
the  pulmonary  artery,  and  its  great  muscular  development  about  the 
origin  of  the  aorta,  where  it  normally  undergoes  involution.  The  effect 
would  be  the  pulling  round  of  the  aorta  at  its  orifice  to  the  position 
normally  occupied  by  the  pulmonary  artery,  so  that  the  relation  of  the 
two  vessels  to  the  auricular  canal  becomes  reversed. 

A  similar  result  would  be  attained  by  supposing  a  reversal  of  the 
normal  right  to  left  bulboventricular  bend,  so  that  this  undergoes  a 
left  to  right  twist,  which  would  bring  the  vessel  lying  posteriorly  into 
relation  with  the  right  side  of  the  auricular  canal.  Such  a  reversal  of 
the  bulboventricular  bend  has  been  suggested  to  me  by  Dr.  Lewis  as  a 
probable  explanation  of  those  cases  of  transposition  in  which  the  trans- 
posed aorta  is  cut  off  by  an  anomalous  septum  from  a  common  ventricle,, 
and  has  also  been  mentioned  in  this  connection  by  Keith. 

From  a  consideration  of  the  above  theories,  and  from  the  study  of  two 
models  made  by  Dr.  Frederic  Lewis  and  myself,  the  series  of  events 
occurring  in  transposition  appears  to  us  to  be  as  follows:  (1)  A  reversal 
of  the  bulboventricular  bend,  so  that  the  two  great  trunks  resulting  from 
division  of  its  distal  aortic  portion  come  to  occupy  a  reversed  relation 
to  the  auricular  canal;  (2)  a  reversal  of  the  normal  kinking  of  the 
aortic  bulb  so  that  it  remains  a  straight  tube  or  assumes  a  curve 
complementary  to  the  reversed  bulboventricular  twist  below;  (3)  a 
consequent  reversal  of  the  normal  spiral  arrangement  of  the  bulbar 
endocardial  ridges;  and  (4)  a  resulting  malposition  of  the  aortic 
septum  so  that  its  spiral  twisting  either  does  not  take  place,  or 
takes  place  in  a  reversed  direction,  leading  to  a  lack  of  the  normal  torsion 
of  the  great  arterial  trunks,  that  is,  transposition. 

The  interdependence  of  the  first  and  second  of  the  above  events  is 
evident  from  a  glance  at  the  compensatory  curves  of  the  normal  embryonic 
bulbus  (Plate  V),  but  the  question  as  to  which  of  these  two  is  of 
primary  occurrence  is  not  altogether  clear.  The  reversal  of  the  bulbo- 
ventricular twist  may  be  of  the  nature  of  a  localized  situs  inversus  and 
the  causative  factor  of  the  whole  proceeding.  Or  it  may  be  secondary 
to  a  primary  arrest  of  the  kinking  that  normally  takes  place  in  the  aortic 
bulbus,  and  of  the  consequent  lack  of  spiral  disposition  of  its  valves. 


CONGENITAL  CARDIAC  DISEASE 


383 


That  the  second  of  these  two  alternatives,  namely,  a  primary  arrest  of 
development,  may  be   the   correct   solution  is  suggested   in  a  striking 


Fig.  35 


A 


\ 


K- 


B  C 

A,  Wenner's  case  of  cor  biatriatrum  triloculare  with  transposed  aorta  and  pulmonary  artery  passing 
up  to  the  right,  and  both  right  and  left  auricular  appendages  displaced  to  the  lett  side.  A,  Superior 
vena  cava;  B,  right  pulmonary  artery;  C,  right  auricle;  D,  ductus  botalli;  E,  left  pulmonarj'  artery; 
F,  right  auricular  appendix;    G,  left  auricular  appendix. 

B,  figure  of  normal  embryonic  heart  sho'wing  the  position  which  the  truncus  comes  to  occupy  between 
the  auricular  appendages  after  the  normal  shunting  to  the  left  of  this  structure  has  taken  place.  A, 
common  arterial  trunk;  B,  B,  auricular  appendages. 

C,  figure  of  embryonic  heart  in  which  the  normal  shunting  to  the  left  of  the  common  arterial  trunk 
has  not  occurred,  so  that  this  structure  still  comes  off  entirely  from  the  right  side  of  the  heart  (as  in  the 
very  early  stages),  and  both  auricular  appendages  still  lie  on  the  left  (as  in  Wenner's  case).  A,  common 
arterial  trunk;  B,  auricular  appendages.  From  Beitrdge  zur  Lehre  der  Herzmisshiidungen,  Case  9. 
Otto  Wennej-,  Virch.  Arch.,  1909,  196,  pp   140,  155, 


384  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

way  by  two  remarkable  cases  reported  by  Wenner/  and  Birmingham^ 
of  "  piire"dextrocardia,  in  which  the  transposed  vessels  arose  from  the 
extreme  right  side  of  a  common  (in  Birmingham's  case  of  a  right)  ventricle, 
and  both  auricular  apjiendages  lay  entirely  on  their  left  side,  just  as  in 
the  very  early  embryo,  the  common  auricle  lies  to  the  left  of  the  common 
trunk.  In  both  cases  the  dextrocardia  was  not  a  true  situs  inversus, 
but  the  apex  of  the  heart  was  formed  by  the  right  ventricle,  again  evi- 
dencing a  persistence  of  an  early  embryonic  state,  and  a  true  primary 
arrest  of  development. 

Pathology. — It  is  impossible  to  follow  Rokitansky's  minute  classifica- 
tion in  a  statistical  study  of  recorded  cases,  for  the  relation  of  the  vessels 
to  each  other  is  often  indefinitely  stated.  For  practical  purposes  the 
classification  into  complete,  corrected,  and  partial  transposition,  suggested 
by  Vierordt,  may  be  used. 

(a)  In  coiii'plete  transposition,  the  vessels  arise  from  reversed  ventricles. 
This  occurred  in  43  of  our  70  cases.  In  18  of  these  the  aorta  arose  from 
the  right  ventricle  to  the  right  and  in  front,  and  the  pulmonary  artery 
to  the  left  and  behind.  The  so-called  transpositio  vera,  in  which  the 
aorta  arises  in  exactly  reversed  relation  to  the  left  and  anteriorly,  and 
the  pulmonary  artery  from  the  left  ventricle  to  the  right  and  posteriorly, 
is  illustrated  by  the  cases  of  Pye-Smith^  and  Thiele.  The  pulmonary 
rose  above  a  defect  in  the  septum  and  the  aorta  from  the  infundibulum 
of  the  right  ventricle  in  three  of  Theremin's  cases,  and  in  those  of  Lees 
and  Rheiner.  The  aorta  rose  from  both  ventricles  above  the  defect, 
the  pulmonary  from  the  left  ventricle,  in  that  by  Buchanan.^ 

(6)  In  corrected  transposition  (see  Fig.  33  Scheme  A),  the  relation  of 
the  vessels  to  each  other  is  altered,  but  they  are  placed  in  their  proper 
ventricles  by  the  union  of  the  interventricular  septum.  Minor  degrees 
of  displacement  probably  often  pass  unnoticed,  for  the  "correction" 
prevents  pathological  results.  More  extreme  grades  can  be  at  once 
recognized.  Six  almost  identical  cases  are  described  by  Rokitansky 
(two  cases),  Rauchfuss,  Tonnies,^  and  Theremin  (two  cases),  in  which 
the  aorta  and  pulmonary  artery  are  completely  reversed  in  relation  to 
each  other,  but  arise  each  from  their  own  ventricle.  In  all  there  was  a 
defect  in  the  interventricular  septum  at  the  base,  and  in  all  the  auriculo- 
ventricidar  orifices  were  also  transposed,  the  mitral  lying  in  the  right,  the 
tricuspid  in  the  left  ventricle,  and  thus  the  "correction"  of  the  transposi- 
tion of  the  arteries  by  the  septum  was  seemingly  nullified;  the  aorta 
arises  from  a  ventricle  of  venous  form  (in  that  it  has  a  tricuspid  valve), 
the  pulmonary  from  an  arterially  constructed  one.  Rokitansky  suggests 
that  in  these  cases  "the  ventricle  in  which  the  septum  arises  anteriorly 
forms  as  the  arterial  one."  Fingerhuth  described  a  case  of  corrected 
transposition  with  situs  inversus  of  the  viscera,  and  no  transposition 
of  the  ventricles. 

1  Virchoivs.  Arch.,  1909,  cxcvi,  127. 

2  Jour.  Anat.  and  Physiol.,  1893,  xxvii,  139. 
^  Trans.  Path.  Soc,  London,  1873,  xxiii,  80. 
4  Ibid.,  1857,  viii,  149. 

^  GoUingen  Thesis,  1884. 


CONGENITAL  CARDIAC  DISEASE  385 

(c)  Partial  transposition,  in  which  both  vessels  arise  from  the  same 
ventricle  or  from  a  common  ventricle  in  reversed  relations,  is  relatively 
infrequent.  Both  vessels  may  arise  from  the  right  ventricle,  as  in  Theremin's 
forty-first  observation  and  in  Tooth's  case/ in  which  a  large  thick-walled 
aorta  arose  from  the  usual  origin  of  the  pulmonary  artery,  which  was 
itself  small,  thin-walled,  bicuspid,  and  was  given  off  from  the  right 
ventricle  directly  behind  the  aorta.  Both  vessels  may  spring  from  the 
left  ventricle.  Thus,  Crocker  reported  a  girl,  aged  thirteen  years,  in 
whom  the  pulmonary  artery,  small  and  constricted,  arose  from  a  small, 
thick-walled,  left  ventricle  to  the  right  and  anteriorly,  while  the  aorta 
arose  from  the  same  cavity  posteriorly,  and  communicated  with  the  right 
ventricle  through  a  defect  in  the  septum.  Both  vessels  may  arise  transposed 
from  a  covimon  ventricle,  as  in  two  examples  of  cor  biatriatrum  triloculare 
with  pulmonary  atresia  and  transposition,  and  in  the  biloculate  heart 
reported  by  Rudolf.  The  remarkable  cases  of  displacement  of  both 
auricular  appendages  to  the  left  of  the  transposed  vessels  fall  in  this 
category.  Finally,  the  transposed  vessels  may  arise  from  a  rudimentary 
cavity  cut  off  by  an  anomalous  septum  from  the  common  ventricle. 

The  condition  of  the  semilunar  cusps  in  transposition  is  of  interest. 
In  one  or  other  of  the  vessels,  more  commonly  in  the  pulmonary,  they 
are  frequently  deformed,  of  unequal  length,  bicuspid,  or,  as  in  Bokay's 
case,  markedly  increased  in  depth.  Their  position  varies  with  the 
degree  of  displacement,  and  should  therefore  be  carefully  observed, 
as  the  degree  of  deviation  may  be  thus  detected.  In  true  transposition, 
for  instance,  the  non-coronary  cusp  in  the  aorta  lies  anteriorly  instead 
of  posteriorly. 

Changes  in  the  relative  size  and  thickness  of  the  tivo  great  trunks  are  usually 
present,  and  are  of  importance  as  supporting  Rokitansky's  view  that 
an  altered  position  of  the  aortic  septum  is  a  fundamental  part  of  the  con- 
dition. In  spite  of  the  fact  that  the  pulmonary  arises  from  the  left 
ventricle,  which  is  anatomically  constructed  as  the  strongest  of  the  two 
chambers,  this  vessel  is  usually  thin-walled  and  narrowed  and  its  orifice 
is  stenosed  or  atresic,  while  the  aorta  is  dilated.  Among  Rokitansky's 
18  cases  of  transposition,  pulmonary  stenosis  or  atresia  occurred  11  times, 
and  it  was  present  in  17  out  of  the  25  cases  of  Keith's  series.  In  a  few 
instances  the  reverse  holds  good  and  a  large  thick-walled  pulmonary  may 
be  combined  with  a  short,  narrow  aorta.  Theremin's  series  of  14  cases 
in  infants,  10  of  which  were  of  complete  and  4  of  partial  transposition, 
forms  a  remarkable  exception  to  the  above  statement.  In  3  of  his  cases 
the  pulmonary  was  dilated,  and  in  the  remainder  it  was  equal  in  size  to 
the  aorta.  In  our  own  series,  which  includes  these  14  of  Theremin's, 
among  70  cases,  in  43  of  which  the  transposition  was  complete,  in  22 
partial,  and  in  5  "corrected,"  the  pulmonary  was  stenosed  or  atresic  in  23 
cases. 

The  Fetal  Passages.- — In  complete  transposition  the  venous  blood 
from  the  right  heart  is  distributed  to  the  arterial  system  through  the 
aorta,  while  the  aerated  blood  entering  the  left  auricle  is  returned  again 

1  Trans.  Path.  Soc,  London,  1879,  xxxi,  92. 
VOL.  IV — 25 


386  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

to  the  lungs  by  the  pulmonary  artery.  The  conditions  of  the  circulation 
are  thus  of  the  poorest,  and  unless  one  or  other  of  the  fetal  passages 
remains  open,  life  cannot  be  sustained.  The  interventricular  septum 
is  frequently  entire,  but  a  widely  patent  foramen  ovale  is  nearly  always 
present  and  combines  with  a  patent  ductus  or  with  a  septal  defect  to 
allow  of  the  passage  into  the  aorta  of  the  aerated  blood.  Very  rarely 
does  one  of  these  conditions  exist  singly.  In  our  series,  among  37  cases 
of  complete  transposition  the  foramen  ovale  was  patent  32  and  the  ductus 
arteriosus  22  times.  A  patent  foramen  was  the  only  communication 
between  the  two  sides  of  the  heart  in  8  cases,  namely,  those  by  Emanuel, 
Doming,^  Kelly,  Cockle,  Bokay,  and  in  three  of  Theremin's  cases.  It 
was  combined  with  a  patent  ductus  in  14  cases,  with  a  defect  of  the  inter- 
ventricular septum  in  6,  and  with  patency  of  both  of  these  openings  in 
4  cases.  The  ventricular  septum  was  completely  closed  in  25  cases  and 
was  defective  in  12,  in  one  of  which,  that  by  Heuyer  and  Campergne,^ 
the  septal  defect  was  the  only  communication,  and  in  another  by  Gutt- 
mann^  the  ductus  arteriosus  was  also  patent.  In  Theremin's  thirty- 
seventh  observation  and  in  Ramm's  case,  a  large  patent  ductus  was  the 
only  communication  between  the  right  and  left  heart.  In  a  few  cases 
the  bronchial  arteries  were  markedly  dilated. 

Bokay*  has  made  an  analysis  of  43  cases  of  complete  transposition, 
with  regard  to  the  condition  of  the  fetal  passages,  and  there  are  33  addi- 
tional cases  in  our  series  from  the  literature.  The  following  table  shows 
the  condition  of  the  fetal  passages  in  these  76  cases. 

F.  0.  Patent.     D.  A.  Patent.     V  S.  Defect. 

F.  O.  patent 16  36  7 

D.  A.  patent 36  3  3 

V.  S.  defect 7  3  5 

Patent  F.  O.,  defect  V.  S.,  patent  D.  A.    ...  4  4  4 

Total      63  46  19 

The  abbreviations  are  as  follows:  F.  0.,  foramen  ovale;  D.  A.,  ductus  arteriosus; 
V.  S.,  interventricular  septum. 

Hypertrophy  and  Dilatation  of  the  Heart. — Under  the  altered  conditions 
of  the  circulation  the  aorta  is  required  to  supply  blood,  not  only  to  the 
systemic  circulation,  but  through  one  or  other  of  the  persistent  fetal 
passages  to  the  lung.  The  right  heart  is  practically  invariably  hyper- 
trophied  and  dilated,  sometimes  to  an  enormous  extent,  and  the  right 
auricle  likewise.  The  left  chambers  usually  share  in  these  changes  though 
to  a  less  degree. 

Symptoms  and  Physical  Signs. — During  the  period  of  fetal  circulation, 
transposition  of  the  vessels  is  of  little  pathological  significance,  so  that, 
unless  associated  anomalies  exist,  the  subjects  are  born  at  full  term,  well 
developed,  and  apparently  normal.     In  complete  transposition,  marked 

1  Trans.  Am.  Fed.  Soc,  1891,  ii,  46. 

2  Bull,  de  la  Soc.  Anat.,  April,  1913,  p.  209. 

3  Deut.  med  Woch.,  1893,  xc,  74. 

4  Arch.f.  Kinderheilk.,  1911,  Iv,  321. 


CONGENITAL  CARDIAC  DISEASE  387 

cyanosis  is  almost  always  a  prominent  feature,  but  it  may  not  be  present 
at  birth,  appearing  usually  after  some  days  or  weeks,  and  perhaps  develop- 
ing, as  Theremin  suggests,  as  the  ductus  becomes  obliterated.  On' 
account  of  the  extreme  degree  of  the  cyanosis,  clubbing  of  the  fingers  and 
toes  usually  develops  in  infants  which  have  survived  the  first  six  months 
of  life.  In  partial  transposition,  on  the  other  hand,  or  in  complete  trans- 
position with  large  septal  defects,  cyanosis  may  be  quite  moderate  in 
degree.  In  Theremin's  patient,  dying  at  three  and  a  half  years,  there 
was  no  cyanosis  until  the  last  illness,  but  large  defects  of  interauricular 
and  interventricular  septa  combined  to  relieve  the  situation.  In  a 
remarkable  case  of  partial  transposition,  recorded  by  Lebert,^  death 
occurred  from  failing  compensation,  at  the  age  of  twenty,  without  any 
sign  of  cyanosis  having  developed  during  life.  The  patient  was  a  young 
man  in  good  health  until  three  years  previously,  when  cardiac  symptoms 
developed  suddenly  after  lifting  a  heavy  weight.  The  aorta  arose  in 
front  of  the  pulmonary  from  the  right  ventricle,  the  pulmonary  was 
stenosed  and  a  large  septal  defect  admitted  the  index  finger. 

In  uncomplicated  cases,  and  where  a  ventricular  septal  defect  is  either 
not  present  or  is  of  small  size,  physical  examination  may  yield  no  evidence 
of  the  defect,  except  a  sharply  accentuated  second  sound  over  the  pulmo- 
nary (aortic)  area.  The  combination  of  marked  cyanosis  with  signs  of 
hypertrophy  of  the  right  heart  and  an  entire  absence  of  adventitious 
sounds,  or  precordial  thrill,  in  an  infant  or  young  child,  is  strongly  sug- 
gestive of  transposition,  and  a  successful  diagnosis  has  frequently  been 
made  on  these  features.  In  Theremin's  thirtieth  observation  this  was 
based  upon  "cyanosis  increasing  when  the  infant  cried,  hypertrophy  of 
the  heart  both  in  vertical  and  transverse  diameter,  the  heart  sounds  loud 
and  accentuated  but  pure,  the  aortic  and  pulmonary  sounds  distinct." 
In  Ramm's  case,  aged  fifty-six  days,  a  probable  diagnosis  was  made. 
Here  also  there  was  cyanosis  from  birth,  no  murmur,  no  accentuation 
or  reduplication  of  the  heart  sounds,  but  dulness  extending  beyond  the 
right  sternal  border  and  upward  to  the  second  rib,  of  so  marked  a  char- 
acter that  a  mediastinal  tumor  was  at  first  suspected.  In  Theremin's 
thirtieth,  thirty-first,  thirty-eight,  and  thirty-ninth  observations  the 
heart  sounds  were  free  from  murmurs,  although  muffied,  and  cardiac 
dulness  was  increased  to  the  right.  On  the  other  hand,  a  loud  systolic 
murmur  with  maximum  intensity  at  the  apex  was  heard  in  Kelley's 
case,  the  same  at  the  base  and  at  the  back  in  Pye-Smith's,  and  was  prob- 
ably produced  by  the  patent  foramen  ovale  present. 

The  patent  ductus,  patent  foramen,  or  septal  defect  present,  may 
produce  their  characteristic  physical  signs,  and  thus  obscure  the  negative 
character  of  the  auscultatory  phenomena,  which  is  significant  of  the 
clinical  picture  of  an  uncomplicated  transposition. 

Prognosis. — The  duration  of  life  varies  in  the  three  groups  distinguished. 
In  complete  transposition  it  is  usually  very  short.  In  32  of  the  40  cases 
in  our  series  it  varied  between  eleven  months  and  a  few  days.  When  a 
septal  defect  is  present,  life  may  be  considerably  prolonged;  the  age  of 

^  Virchows  Arch.,  1863,  Ixxxii,  405. 


388  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

sixteen  years  was  attained  by  Keith's  patient,  eleven  by  Emanuel's, 
and  four  by  Buchanan's.  In  partial  or  corrected  transposition  on  the 
other  hand,  early  adult  life  is  usually  reached.  Birmingham's  patient 
was  twenty,  Tonnies'  twenty-one,  Elliott's^  nineteen.  Young's  thirty- 
six,  and  Geipel's  forty-six  years.  Vierordt  gives  the  following  analysis 
of  the  duration  of  life  in  75  cases.  To  this  is  added  that  of  the  70  in 
our  series,  of  which  there  were  57  classified  as  the  primary  lesion,  and  13 
complicating  other  defects. 

Duration  of  Life  in  Transposition 

Cases  in  this  series.  Vierordt's 

Age.  Complete      Partial.     Corrected.     Total.  cases. 

Born  dead 0  0  0  0  3 

1  to  24  hours  .......  0  0  0  0  1 

1  to    7  days 0  0  0  0  7 

7  to  14     "           7  2  0  9  5 

14  to  30     "           3  2  0  5  7 

1  to    2  months 8  0  0  8  14 

2to    6     "           7  2  2  11  12 

6  to  12     "           7  1  0  8  9 

1  to    2  years 1  2  0  3  2 

2  to    5     "           6  0  0  6  6 

6  to  10     "           1  2  0  3  0 

10  to  11     "  .1  1  0  2  3 

11  to  21     "  1  6  0  7  0 

21  to  30     "  1  0  0  1  5 

30  to  40     "  0  2  2  4  1 

40  to  50     "  0  0  1  1  0 

Age  nnmentioned 0  2  0  2  0 


Total 43  22  5  70  75 


PULMONARY  STENOSIS  AND  ATRESIA. 

Pulmonary  stenosis  is  the  form  of  cardiac  defect  most  familiar  to  the 
practitioner.  It  is  of  much  clinical  importance  on  account  of  its  com- 
parative frequency,  the  relatively  long  duration  of  life,  and  the  prominence 
of  the  cyanosis  nearly  always  associated.  To  the  student  also  it  is  a  sub- 
ject of  the  highest  interest,  for  in  its  symptomatology  and  pathogenesis 
are  focused  the  most  difficult  problems  of  congenital  cardiac  disease. 

Owing  to  the  wide  variations  in  the  conditions  presented  and  the 
differing  aspects  from  which  the  subject  must  be  approached,  a  classifica- 
tion of  the  different  forms  is  as  difiicult  as  it  is  necessary.  Rauchfuss 
points  out  that  the  simple  anatomical  findings  furnish  the  best  guide  to 
a  useful  grouping.  Thus,  the  degree  of  narroicirig_is  important,  and  a 
simple  stenosis  is  to  be  distinguished  from  a  complete  atresia;  from  the 
stand-point  of  pathogenesis,  the  seat  and  character  of  the  stenosis  are 
criteria  of  much  value;  and  thirdly,  the  presence  or  absence  of  defects 
of  the  mterventricidar  seiDtum  provides  a  dividing  line  of  the  greatest 
importance.  This  last  is  important  etiologically  as  indicating  the  stage 
of  embryonic  or  fetal  life  at  which  the  stenosis  took  place,  and  clinically 
in  that  the  duration  of  life  and  symptomatology  differ  somewhat  in  the 
two  groups. 

1  Jour.  Anat.  and  Physiol.,  1877,  xi,  302. 


CONGENITAL  CARDIAC  DISEASE  389 

Statistics. — Pulmonary  stenosis  is  probably  the  commonest  of  all 
oarcW^r  anomaHls.  The  oases  are  scalfer'eJ'soTreerv'-TErougK  the  literature 
that  an  exact  statistical  statement  is  impossible.  Vierordt  estimated  at 
least  300  in  1898,  and  placed  coarctation  of  the  aorta  next  in  frequency 
with  130  cases.  Among  181  anomalies  of  the  heart  which  he  analyzed, 
Peacock  found  119  of  pulmonary  defect.  In  1906  Keith^  examined  185 
specimens  of  cardiac  malformations  in  the  hospital  museums  of  London, 
and  found  that  in  135,  or  70  per  cent.,  there  was  an  anomalous  con- 
dition of  the  pulmonary  tract,  the  deformity  being  in  the  conus  of 
the  right  ventricle  in  133  cases  and  in  the  pulmonary  valve  in  22. 
In  his  later  communication,^  among  272  malformed  hearts  examined 
in  the  various  London  Museums,  Keith  found  141  in  which  the  defect 
was  due  to  an  imperfect  transformation  of  the  bulbus  cordis  of  the 
embryo.  Of  these  141,  in  19,  there  was  incomplete  fusion  of  the 
infundibulum  with  the  body  of  the  right  ventricle  (conus  a  separate 
chamber) ;  in  44,  partial  arrest  in  development  of  the  infundibulum ;  in 
37,  complete  arrest  of  the  infundibulum;  in  23  fusion  of  semilunar  valves; 
in  7,  partial  or  complete  absence  of  the  body  of  the  right  ^'entricle  with 
development  of  the  infundibulum;  in  4  subaortic  stenosis;  and  in  7, 
congenital  aortic  stenosis. 

Among  the  cases  of  anomalies  analyzed  here,  there  are  150  of  congenital 
pulmonary  disease.  The  proportion  of  stenosis  to  atresia  in  recorded 
cases  analyzed  is  as  follows: 

Number 
Stenosis.      Atresia.       analyzed. 

Kussmaul 64  26  90 

Rauchfuss 81  33  114 

Peacock 90  29  119 

Vierordt 83  24  107 

Theremin 20  10  30 

This  series .116  34  150 

The  relatively  high  percentage  of  atresia  in  Theremin's  cases  is  ex- 
plained b}"  the  fact  that  his  material  was  entirely  among  infants,  in  whom 
the  mortality  from  atresia  is  high. 

The  condition  of  the  fetal  passages  was  the  subject  of  statistical  study 
by  Meyer,  Kussmaul,  Taruffi,  and  other  authors,  of  whose  work  a  full 
review  is  given  by  Vierordt.  A  defect  of  the  interventricular  septum 
exists  in  the  great  majority.  'THe  number  of  cases  with  closed  septum 
IS  relatively  larger  in  atresia  than  in  stenosis;  thus  Rauchfuss  finds 
among  192  cases,  171  in  which  the  interventricular  septum  is  defective 
and  21  in  which  it  is  closed.  Of  these  21,  10  are  cases  of  atresia  and  11  of 
stenosis.  Among  Vierordt's  83  cases  of  stenosis  are  71  with  defective 
and  12  with  closed  interventricular  septum;  among  his  24  of  atresia,  in 
14  the  septum  was  defective  and  in  10  it  w^as  closed.  When  the  inter- 
ventricular septum  is  entire,  the  foramen  ovale  is  usually  widely  patent, 
but  it  also  may  in  rare  cases  be  closed. 

Among  the  116  cases  of  stenosis  analyzed  here  the  interventricular 
septum  was  defective  in  95,  and  entire  in  21  cases.     In  12  of  these  21 

1  Festschrift,  Quater centenary  Aberdeen  University,  July,  1906. 

2  Hunterian  Lectures,  Lancet,  1909,  ii,  359,  433,  519. 


390  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

the  foramen  ovale  was  patent,  but  in  9  the  auricular  septum  was  also 
closed,  and  no  communication  existed  between  the  two  sides  of  the 
heart.  Among  the  34  cases  of  atresia  the  interventricular  septum  was 
defective  in  26  and  entire  in  8  cases.  Of  these  8,  in  one  the  foramen 
ovale  was  also  closed  but  there  was  a  large  patent  ductus.  The  ductus 
arteriosus  is  nearly  always  patent  in  atresia  but  is  usually  closed  in 
jtenosis.  Among  the  82  cases  of  pulmonary  stenosis  classed  as  the  pri- 
mary lesion  in  the  chart,  the  ductus  was  patent  in  only  11;  of  the  24  cases 
of  atresia,  it  was  patent  in  15  cases.  The  condition  of  the  cardiac  septa 
and  ductus  in  these  cases  was  as  follows: 

Stenosis.  Atresia. 

Number         Number  with      Number      Number  with 
analyzed.        patent  D.  A.      analyzed,     patent  D.  A. 

F.  0.  and  V.  S.  closed       ...  7  0  1  1 

F.  O.  patent,  V.  S.  closed       .      .  11  1  5  4 

F.  O.  closed,  defect  V.  S.  ...  40  5  7  3 

F.  O.  patent,  defect  V.  S.  ...  24  5  11  7 

Total 82  11  24  15 

Rechtslage  of  the  aorta  is  present  in  the  majority  of  cases  with  septal 
defect,  and  is  especially  frequent  in  atresia.  Among  the  64  cases  of 
pulmonary  stenosis  with  septal  defect,  classified  in  the  chart  as  the 
primary  lesion,  it  was  present  in  39  cases,  in  29  of  which  the  aorta  arose 
from  both  ventricles  above  the  defect,  and  in  10  chiefly  or  entirely  from 
the  right  ventricle.  Among  the  18  cases  of  atresia  with  septal  defect 
the  aorta  arose  from  both  ventricles  above  the  defect  in  5,  entirely  from 
the  right  ventricle  in  10. 

Pathology. — Pulmonary  Stenosis. — The  narrowing  may  involve  the 
whole  pulmonary  tract,  or  be  localized  to  the  valve,  artery,  or  conus. 
Two  distinct  types  may  be  recognized: 

1.  In  a  few  cases  the  stenosis  is  valvular  in  character  and  is  produced 
by  a  thickening,  shortening,  or  fusion  of  the  pulmonary  cusps.  A  thick 
diaphragm  with  three  raphe  of  fusion  on  its  arterial  surface  is  usually 
formed,  which  protrudes  into  the  pulmonary  artery  in  a  funnel-shaped 
way  and  is  perforated  by  a  circular  or  triangular  opening  of  varying  size. 
The  pulmonary  artery  is  frequently  dilated  above,  may  be  normal,  or 
somewhat  thin-walled.  The  conus  below  the  valve  shares  in  the  hyper- 
trophy of  the  right  ventricle,  but  is  otherwise  normal;  the  interventricular 
septum  is  usually  closed.  There  is  every  evidence  to  show  that  the 
stenosis  has  originated  in  an  inflammatory  process,  in  later  fetal  life  after 
the  heart  has  been  fully  formed.  ^' 

2.  In  the  second  and  larger  group,  the  cusps  may  or  may  not  be 
thickened  or  fused,  but  the  stenosis  is  due  to  a  rudimentary  condition, 
hypoplasia,  or  deformity  of  some  part  of  the  pulmonary  tract.  In 
these  cases  a  defect  of  the  interventricular  septum  is  usually  associated 

"^and  a  deviation  to  the  right  of  the  aorta,  so  that  this  arises  from  both 
ventricles  above  the  defect,  or  chiefly  from  the  right  ventricle,  communica- 
ting with  the  left  through  the  defect.  Such  forms,  which  suggest  a 
'developmental  origin,  make  up  the  great  majority  of  cases  of  pulmonary 
stenosis,   and  the   combination  of  these  three   conditions,   pulmonary 


CONGENITAL  CARDIAC  DISEASE  391 

[stenosis,  defect  of  the  septum  at  the  base,  and  rechtslage  of  the  aorta,/ 

I  is  probably  the  commonest  of  all  cardiac  anomalies. 

In  the  majority  of  cases — in  Keith's  estimate  90  per  cent. — the  conns 
of  the  right  ventricle  is  involved  in  the  deformity.  Two  distinct  types  of 
conus  stenosis  may  be  distinguished.  The  whole  infundibulum  may  be 
more  or  less  constricted,  its  musculature  thickened,  and  th^  endocardium 
opaque.  In  a  case  of  this  kind,  reported  by  Cautley,^  the  pulmonary 
cusps  were  delicate  and  healthy  above  the  stenosis,  but  both  they  and  the 
artery  were  very  small.  Usually  the  valves  are  thickened,  fused,  or  rudi- 
mentary, and  they  are  often  bicuspid.  Sometimes  a  thin  diaphragm 
with  delicate  raphe  showing  no  sign  of  inflammatory  change,  is  stretched 
across  the  pulmonary  orifice,  suggesting  an  incomplete  division  of  the 
endocardial  cushions.  In  these  cases  a  defect  of  the  septiun  is  almost 
invariably  present. 

A  second  group  of  conus  deformities  is  that  in  which  a  cavity,  described 
by  some  of  the  older  pathologists  as  a  third  ventricle,  is  cut  off  from 
the  sinus  of  the  right  ventricle  by  a  definite  septum  perforated  by  a 
small  opening.  There  are  19  in  this  series,  including  one  case  of  atresia. 
Keith  describes  and  figures  an  illustration  of  this  anomaly.  The  infundi- 
bulum is  enormously  dilated  and  communicates  with  the  sinus  by  a 
small  opening  with  thickened  fibrous  borders.  The  pulmonary  cusps 
are  large  and  competent,  the  artery  dilated  above,  and  there  is  a  small 
defect  of  the  interventricular  septum.  Another  variation  of  this  form 
of  conus  deformity  is  figured  by  Andrewes.^  The  conus  is  atrophied  and 
is  represented  by  a  small  cavity  with  thick  muscular  walls.  It  com- 
municates with  the  sinus  of  the  ventricle  by  an  opening  admitting  a 
crow-quill  one-quarter  inch  below  the  pulmonary  cusps,  which  are 
small,  bicuspid,  and  not  thickened. 

1     Hypertrophy  and  dilatation  of  the  right  ventricle  and  auricle  are 

/constant  in  pulmonary 'stenosis,  and  in  the  cases  associated  with  defect- 
of  the  septum  at  the  base  and  rechtslage  of /the  aorta.  In  these  the 
hypertrophy  is  sometimes  most  marked  in  the  wall  of  the  sinus  of  the 
ventricle,  indicating  that  it  had  been  produced  by  the  force  needed  to 
send  the  blood  into  the  aorta  through  the  defect,  rather  than  by  the 
obstruction  in  the  pulmonary  artery.  The  aorta  is  usually  thick  walled 
and  of  large  caliber.  In  the  developmental  cases  the  pulmonary  artery 
is  usually  narrow  and  thin-walled,  resembling  a  vein  in  structure.  When 
the  stenosis  is  confined  to  the  valve,  the  artery  may  be  dilated. 

Pulmonary  Atresia. — All  that  has  been  said  of  the  seat  and  character  of 
the  deformity  in  pulmonary  stenosis  applies  equally  to  a  complete  atresia. 
In  a  small  series  of  cases  the  point  of  obliteration  is  at  the  valve,  the 
artery  dilated  above,  the  foramen  ovale  widely  open,  and  the  septum 
entire.  In  Weiss'^  case  the  seat  of  atresia  was  in  the  conus,  which  admitted 
only  a  pinhead  or  a  fine  straw,  and  was  lined  by  thickened  endocardium; 
just  above  this  were  two  fairly  large  pulmonary  cusps,  and  the  artery 
itself  was  comparatively  large.    There  was  a  small  patent  foramen  ovale, 

1  Edin.  Med.  Jour.,  1902,  xii,  257.  2  Trans.  Path.  Soc,  1865,  xvii,  45. 

s  Brit.  Med.  Jour.,  1877,  ii,  378. 


392 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


a  large  defect  of  the  septum  at  the  base,  and  a  large  thick-walled  aorta 
arose  frora  the  right  ventricle  above  the  defect. 

The  pulmonary  artery  may  be  obliterated  for  some  distance  above  the 
valve,  forming  a  fibrous  cord,  which  may  emerge  suddenly  from  the  fleshy 
outer  wall  of  the  ventricle  and  give  no  sign  of  its  origin  from  within,  or  it 
may  be  patent  throughout,  diminishing  toward  the  orifice  in  a  funnel- 
shaped  way.  In  such  cases  the  cusps  may  be  seen  thickened  and  fused 
with  each  other  at  the  bottom  of  the  cul-de-sac  formed  by  the  artery, 
or  they  may  form  a  triradiate  elevation  of  three  fleshy  cushions;  or  no 
trace  of  them  may  remain.  The  aorta  is  usually  very  large.  When  a 
defect  in  the  septum  is  present  it  rides  over  it,  or  in  many  cases  arises 
entirely  from  the  right  ventricle. 


Fig.  36 


Heart  and  lungs  of  an  infant,  cyanotic  from  birth,  showing  (A)  atresia  of  the  pulmonary  artery;  B, 
patent  ductus  arteriosus  suppljdng  lungs;  C,  defect  of  interventricular  septum  at  pars  membranacea, 
guarded  by  (D)  an  anomalous  valve  with  false  chordse  tendinese;  E,  tricuspid  orifice;  F,  left  pulmonary 
artery;  G,  right  pulmonary  artery;  H,  cord  passing  through  the  septal  defect;  LA,  left  auricle.  (From 
a  specimen  in  the  McGill  Pathological  Museum,  presented  by  Sir  Wilham  Osier.) 

The  foramen  ovale  is  frequently  widely  patent.  It  may  be  the  only 
means  of  communication  between  the  two  sides  of  the  heart,  the  inter- 
ventricular septum  remaining  entire.  Such  cases  are  less  frequent,  and 
the  condition  is  more  serious  than  that  associated  with  septal  defect. 

The  alterations  in  the  cavities  of  the  heart  vary  with  the  condition 
of  the  interventricular  septum.  When  this  is  entire  the  left  ventricle 
is  greatly  hypertrophied  and  dilated,  and  both  auricles  share  in  the 
enlargement,  while  the  right  ventricle  undergoes  a  true  concentric  hyper- 
trophy, its  wall  becoming  greatly  thickened  and  its  cavity  aplastic  and 


CONGENITAL  CARDIAC  DISEASE  393 

lined  with  opaque  thickened  endocardium,  or  in  some  instances  completely 
obliterated.  When  a  defect  of  the  septum  exists,  the  right  ventricle  is 
greatly  hypertrophied  and  is  dilated  as  well,  and  the  right  auricle  is 
correspondingly  enlarged,  the  left  chambers  remaining  relatively  small. 
When  the  aorta  rides  over  the  defect  in  the  septum,  the  left  ventricle 
may  share  in  the  hypertrophy. 

The  Pulmonary  Circulation. — When  the  pulmonary  artery  is  obliterated 
the  blood  supply  usually  reaches  the  lungs  through  the  widely  patent 
ductus,  but  this  is  sometimes  closed  or  absent.  IMeckel  first  suggested 
that  in  these  cases  the  dilated  bronchial  arteries  might  perform  this 
function,  and  this  is  usually  the  case.  The  lungs  were  supplied  from  the 
left  subclavian  in  Chambers'  case,  from  a  dilated  ductus  arising  from  the 
left  subclavian  in  Ramsbotham's.  In  a  case  reported  by  Voss  two  large 
bronchial  arteries  passed  into  each  lung,  and  were  accompanied  by  an 
anomalous  branch  from  each  coronary.  In  Koller-Aeby's  case  the  ductus 
was  absent,  and  three  large  vessels,  equalling  the  carotid  in  size,  arose  from 
the  upper  thoracic  aorta  at  the  site  of  origin  of  the  bronchial  arteries. 
The  first  turned  to  the  right  lung,  following  the  bronchi ;  the  others  were 
given  ofi^  as  a  common  trunk,  which  divided  into  a  larger  branch  going  to 
the  right  and  a  smaller  to  the  left  lung. 

Pathogenesis. — Two  problems  are  presented.  (1)  Is  the  stenosis  of 
inflammatory  or  of  developmental  origin?  (2)  What  is  the  relation  of  the 
septal  defect  so  often  associated? 

It  must  be  recognized  that  a  small  group  of  cases  occurs  in  which 
the  stenosis  is  strictly  limited  to  the  valves  and  no  septal  defect  is  asso- 
ciated and  which  present  appearances  identical  with  those  produced  by 
the  chronic  valvular  disease  of  postnatal  life.  Such  cases  must  be  sup- 
posed to  be  the  result  of  an  endocarditis  in  later  fetal  life. 

In  the  large  majority  of  cases  a  defect  of  the  interventricular  septum  is 
associated,  indicating  that  if  the  stenosis  be  due  to  an  endocarditis,  this 
must  have  occurred  before  the  development  of  the  heart  was  complete 
at  the  end  of  the  second  month  of  gestation.  It  is  upon  these  cases 
that  the  discussion  really  turns.  It  is  evident  that  if  endocarditis  can 
take  place  during  the  later  stages  of  gestation,  it  may  occur  earlier  as 
well.  On  the  other  hand,  there  are  many  cases  in  which  the  presence  of 
associated  defects  and  the  absence  of  inflammatory  action  show  positively 
that  arrest  of  development  has  been  the  cause.  The  view  is  now  gener- 
ally held  that  in  the  past  far  too  great  weight  has  been  laid  upon  the 
part  which  inflammatory  processes  take  in  the  etiology  of  the  many 
forms  of  pulmonary  stenosis  which  date  back  to  early  embryonic  life, 
before  the  development  of  the  heart  was  completed.  The  theory  of 
fetal  endocarditis  as  a  cause  must  now  be  considered  to  be  of  but 
limited  application.  The  fault  lies  undoubtedly  in  most  cases  in  a 
primary  arrest  of  development. 

Reference  has  been  made  to  the  explanation  communicated  by  Keith,, 
which  is  now  generally  accepted,  that  in  the  majority  of  cases  the  stenosis  j 
is  primary  in  the  conus,  and  is  the  result  of  an  arrest  of  development' 
at  a  stage  when  there  existed  in  the  heart  a  fourth  primitive  chamber, 
the  bulbus  cordis.     In  accordance  with  the  researches  of  Greil,  he  de- 


394  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

scribes  three  changes  as  taking  place  in  the  evolution  of  the  mammalian 
from  the  primitive  heart  of  the  fish  and  reptilia:  (1)  The  division  of  the 
primitive  auricle  and  ventricle;  (2)  the  submerging  of  the  sinus  venosus 
in  the  musculature  of  the  right  auricle;  and  (3)  the  separation  of  the 
bulbus  cordis  from  the  left  ventricle  and  aorta,  and  its  complete  incor- 
poration in  the  right  ventricle  as  the  infundibulum  of  that  chamber. 
This  last  change  takes  place  by  an  upgrowth  of  the  ventricular  mus- 
culature around  the  cavity  of  the  bulbus,  the  musculature  of  the  latter 
being  replaced  by  that  of  the  ventricle,  in  the  same  way  as  the  musculature 
of  the  auricle  replaces  a  great  part  of  that  of  the  sinus  venosus.  The 
author  considers  that  "the  submergence  of  the  bulbus  constitutes  a  critical 
phase  in  the  developmental  metamorphosis  of  the  heart,  and  it  is  during  this 
time  that  malformations  are  apt  to  occur." 

Four  different  types  of  conus  stenosis  are  distinguished  by  him,  of 
which  the  first  is  that  well-differentiated  form  in  which  the  conus  forms 
a  separate  chamber,  being  separated  from  the  sinus  by  a  muscular 
partition.  Peacock,  in  describing  a  similar  case,  compared  it  to  the  three- 
ventricled  heart  of  the  curtle,  and  considered  that  it  represented  an  arrest 
of  development.  Keith  explains  the  condition  as  being  simply  an  arrest 
of  development  in  which  the  infundibulum  and  body  of  the  right  ventricle 
have  developed  to  a  normal  extent,  while  a  constriction  has  remained 
between  them,  representing  the  Dentricular  origin  of  the  bulbus  or  a  per- 
sistence of  the  lower  bulbar  orifice.  The  other  forms  of  conus  stenosis, 
in  which  there  is  a  constriction  more  or  less  complete  of  the  whole 
infundibulum,  he  explains  as  an  arrest  of  development  of  the  bulbus 
as  a  whole,  its  musculature  failing  to  become  submerged  in  that  of  the 
right  ventricle  proper. 

Associated  Anomalies. — Grave  cardiac  defects  are  frequently  asso- 
ciated, especially  in  pulmonary  atresia,  and  constitute  another  argument 
in  favor  of  a  developmental  origin.  In  Ettlinger's  case  there  was  a  large 
defect  in  the  interauricular  septum  above,  with  multiple  defects  of  the 
interventricular  septum,  and  the  pulmonary  veins  opened  into  the  right 
auricle.  In  Habershon's  there  was  false  dextrocardia,  tricuspid  stenosis^ 
defect  of  the  interventricular  septum,  and  horseshoe  kidney. 

A  fact  of  much  importance  is  the  presence  of  associated  anomalies  in 
cases  of  atresia  with  closed  ventricular  septum,  which  might  reasonably 
be  considered  to  be  of  inflammatory  origin.  It  seems  probable  that  the 
primary  condition  here  was  a  narrowing  of  the  conus  or  orifice  in  an 
arrest  of  development,  and  that  the  obliteration  was  produced  by  an 
endocarditis  supervening  in  later  fetal  life. 

Symptoms. — The  majority  of  cases  of  pulmonary  stenosis  and  atresia 
present  the  classical  picture  of  congenital  cyanosis  in  all  its  details.  So 
frequent  is  the  association  between  the  two  conditions,  that  morbus 
cceruleus  and  pulmonary  stenosis  have  been  considered  almost  synony- 
mous terms.  The  clinical  aspects  vary  to  a  certain  extent  with  the  pres- 
ence or  absence  of  defects  of  the  interventricular  septum,  and  with  the 
degree  of  deformity.  In  stenosis  with  closed  septum  cyanosis  is  usually 
slighter  and  of  later  incidence,  and  the  duration  of  life  much  longer. 
The  most  typical  instances  of  congenital  cyanosis  with  bluish  discolora- 


CONGENITAL  CARDIAC  DISEASE  395 

tion  of  the  skin,  becoming  pronounced  on  exertion,  clubbing  of  the  fingers, 
dysnpoea,  and  cyanotic  attacks  are  seen  in  the  many  cases  in  which 
pulmonary  stenosis  is  combined  with  defect  of  the  septum  and  rechtslage 
of  the  aorta.  Pulmonary  atresia  differs  from  a  simple  stenosis  in  the 
more  extreme  degree  of  the  cyanosis.  These  are  the  cases  of  true  morbus 
cceruleiis,  in  which  a  constant  deep  blue,  or  even  purple,  discoloration 
exists,  increasing  to  black  on  violent  exertion.  Here  the  opposite  con- 
dition in  relation  to  septal  defects  is  seen.  When  the  septum  is  closed  the 
cyanosis  is  more  extreme  and  the  duration  of  life  correspondingly  shorter. 
Pulmonary  stenosis  with  defect  of  the  septum  in  which  no  cyanosis  is 
present  is  exceedingly  rare,  but  a  few  cases  are  on  record. 

Physical  Signs  in  Pulmonary  Stenosis. — These  are  generally  distinc- 
tive, but  may  be  obscured  by  those  of  the  septal  defect  so  often  asso- 
ciated. In  typical  cases,  enlargement  of  cardiac  dulness  to  the  right  and 
above,  precordial  bulging,  epigastric  and  precordial  pulsation  indicate  an 
enlargement  of  the  right  heart.  Sometimes  the  cardiac  impulse  may  be 
so  violent  that  the  head  and  neck  share  in  the  vibration  of  the  chest. 
A  thrill,  localized  to  the  second  and  third  left  spaces,  or  diffuse  over  the 
precordium,  is  fairly  frequent.  Its  presence  seems  to  depend  somewhat 
upon  the  condition  of  the  septa.  Rolleston,^  in  reporting  a  case  of  stenosis 
with  rechtslage,  in  which  there  was  no  precordial  thrill,  explains  this  by 
the  presence  of  a  large  defect  of  the  septum,  through  which  the  blood 
current  passed  with  ease  into  the  aorta.  He  says  that  the  evidence  in 
recorded  cases  is  contradictory  upon  this  point,  and  suggests  a  statistical 
study  of  it.  A  thrill  was  present  in  17  of  the  82  "primary"  cases  analyzed. 
In  these  the  condition  of  the  fetal  passages  was  as  follows: 

Cardiac  septa.  Number  analyzed.       Number  with  thrill. 

F.  O.  and  V.  S.  closed 7  "  3 

F.  O.  patent  and  V.  S.  closed     ....  11  4 

F.  O.  patent,  defect  V.  S 24  7 

F.  0.  closed,  defect  V.  S 40  3 

That  is  to  say,  in  more  than  a  third  of  the  cases  with  closed  ventricular 
septum  a  thrill  was  present,  as  also  in  7  of  the  24  in  which  both  foramen 
ovale  and  ventricular  septum  were  opon.  But  in  the  40  cases  with 
closed  foramen  ovale  and  with  defect  of  the  septum  a  thrill  was  absent 
in  all  but  3;  in  one  of  which  there  was  a  large  patent  duct,  which  was 
apparently  the  cause. 

These  figures  are  puzzling  at  first,  but  interesting  on  reflection,  and  are 
large  enough  to  be  of  value  as  facts.  The  inference  is  that  a  thrill  is 
frequently  present  when  the  interventricular  septum  is  entire,  and  also 
when  a  defect  of  that  septum  coexists  with  a  widely  patent  foramen 
ovale;  when  the  interauricular  septum  is  closed  and  the  interventricular 
open  a  thrill  is  rare,  and  when  it  does  occur  may  perhaps  be  ascribed  to  the 
associated  septal  defect.    Further  statistics  are  needed. 

The  pulmonary  second  sound  is  weak  or  absent  in  a  certain  propor- 
tion of  cases.  Much  stress  has  been  laid  upon  the  absence  of  pulmonary 
accentuation  as  a  diagnostic  sign  of  pulmonary  stenosis,  but  in  a  number 
it  has  been  distinctly  louder  than  normal. 

^Trans.  Path.  Soc,  London,  1892,  xliii,  32. 


396  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

A  prolonged,  harsh,  rasping,  or  blowing  systolic  murmur  heard  over  the 
whole  cardia,  but  chiefl}^  at  the  base,  with  its  point  of  maximum  intensity 
over  the  upper  part  of  the  sternum  and  the  second  left  space,  is  present 
in  the  great  majority  of  cases.  It  is  transmitted  upward  toward  the 
clavicle,  along  the  course  of  the  pulmonary  artery,  and  over  the  sternum, 
but  is  faint  or  inaudible  at  the  apex  and  to  the  right  of  the  sternum. 
It  may  be  so  loud  as  to  be  heard  over  the  whole  chest.  From  this  type 
important  variations  occur.  (1)  The  murmur  may  be  heard  over  the 
whole  cardia,  but  with  maximum  intensity  at  the  apex,  as  in  Cassel's 
case,  a  boy  aged  thirteen  years,  w^ith  pulmonary  stenosis  and  a  patent 
foramen  ovale,  but  the  ventricular  septum  entire.  In  a  case  of  Peacock's 
there  was  a  loud  systolic  murmur  over  the  whole  heart  and  along  the 
sternum,  the  maximum  intensity  of  which  was  at  the  inner  side  of,  and 
immediately  above,  the  left  nipple.  In  this  instance  a  septal  defect  was 
associated.  (2)  In  cases  in  which  the  septal  defect  is  present  the  murmur 
may  be  heard  over  the  aortic  area  and  along  the  carotids.  Eisenmenger 
mentions  this  as  a  diagnostic  point  for  the  association  of  pulmonary 
stenosis  with  a  septal  defect.  In  Scheele's^  case,  a  girl  aged  fifteen  years, 
with  marked  cyanosis,  the  pulmonary  orifice  admitted  a  thin  pencil, 
the  valves  were  small  and  shrunken,  the  conus  was  reduced  to  the  size  of 
a  pea,  and  the  septum  was  defective  at  the  base.  There  was  a  systolic 
murmur  along  the  course  of  the  pulmonary  artery  and  at  the  left  sterno- 
clavicular articulation,  which  was  transmitted  far  up  the  carotids  and 
along  both  subclavians,  and  was  most  marked  over  the  left  carotid.  It 
was  heard  also  at  the  aortic  cartilage.  (3)  The  murmur  may  be  heard 
in  the  back,  in  the  left  infrascapular  region.  This  occurred  in  a  number 
of  the  cases  in  this  series,  but  in  all  a  septal  defect  was  associated,  to  which 
the  transmitted  murmur  was  probably  due.  (4)  In  a  few  cases  physical 
signs  are  absent.  Variot  reports  a  child  aged  five  years,  with  a  large 
defect  of  the  septum,  and  the  pulmonary  a  small  thin  cord  with  rudi- 
mentary valves,  who  presented  marked  cyanosis  with  clubbing,  but 
whose  heart  sounds  were  clear. 

Diagnosis. — In  the  majority  of  cases  the  decided  localization  of  murmur 
and  thrill,  the  increased  cardiac  dulness  to  the  right,  the  absence  of  pul- 
monary accentuation,  and  the  presence  of  the  distinctive  symptoms  of 
pronounced  congenital  cyanosis  make  a  positive  diagnosis  possible.  On 
the  other  hand,  the  variation  in  the  character  of  the  murmur  and  of  the 
pulmonary  second  sound,  and  the  occasional  absence  of  cyanosis,  render 
the  diagnosis  indefinite  in  a  certain  proportion  of  cases.  That  such 
atypical  cases  occur  also  makes  it  very  difficult  to  exclude  the  possibility 
of  pulmonary  stenosis  in  the  difi^erential  diagnosis  of  other  cardiac  defects. 
The  presence  of  constant  and  marked  cyanosis,  the  distinctive  character 
of  the  murmur,  and  the  fact  that  it  is  not  usually  heard  in  the  back,  are 
points  in  favor  of  stenosis.  In  patency  of  the  duct,  pulmonary  accentua- 
tion is  the  rule,  in  pulmonary  stenosis  it  is  the  exception. 

Both  the  associated  defect  in  the  interventricular  septum  and  the 
pulmonary  stenosis  have  frequently  been  diagnosed.     The  presence  of 

1  Deut.  med.  Woch.,  1888,  xl,  294. 


CONGENITAL  CARDIAC  DISEASE 


397 


a  thrill  speaks  rather  for  a  closed  septum,  or  for  cases  in  which  the  foramen 
ovale  also  is  patent.  In  a  large  number  of  cases  the  presence  of  the  two 
distinctive  murmurs  can  be  easily  traced,  that  due  to  the  pulmonary 
obstruction  heard  best  at  the  base  and  transmitted  beneath  the  clavicle, 
that  due  to  the  defect  localized  at  the  fourth  space,  heard  also  in  the 
back,  both  harsh,  but  of  the  two,  the  pulmonary  usually  of  a  more 
blowing  character. 

Course. — The  duration  of  life  in  pulmonary  stenosis  with  closed 
interventricular  septum  is  relatively  high.  Peacock  reports  a  patient 
dying  at  forty-five  years,  and  the  lowest  age  in  this  series  was  four  years. 
The  possibility  exists  in  such  cases  that  the  stenosis  had  advanced,  or 
even  originated  since  birth.  In  stenosis  with  septal  defect  death  occurs 
earlier,  but  adult  life  is  also  sometimes  attained.  The  maximum  age  in 
this  series  was  twenty  years.  In  pulmonary  atresia  life  is  very  short. 
The  patients  with  closed  septum  all  die  within  the  first  few  months. 
When  a  defect  of  the  interventricular  septum  exists  these  subjects  may 
live  some  years.  The  highest  age  recorded  was  thirteen  years,  in  one  of 
Peacock's  cases.  The  table  gives  the  duration  of  life  in  the  cases  in  the 
series  in  which  this  point  is  mentioned: 


Pulmonary  Stenosis. 


Age  at  death. 

Before  1  year        .... 

1  to  7  years 

7  to  14  years  

14  to  20  years  .... 
20  to  28  years  .... 
28  to  4.5  years       .... 

Number  of  cases  analyzed 


F.  0.  closed. 

F.O.  patent 

V.  S.  closed. 

defect  V 

s. 

defect  V.  S 

0 

4 

3 

2 

17 

8 

4 

6 

5 

5 

8 

5 

6 

3 

1 

1 

0 

0 

18 


38 


22 


Pulmonary  Atresia. 


Age  at  death. 

In  first  week  . 
1  to  4  weeks  . 
1  to  3  months 
3  to  6  months 
6  to  9  months 

9  to  12  months 
1  to  5  years  . 
5  to  10  years  . 

10  to  13  years 


Number  of  cases  analyzed 


F. 

0 

closed, 

F.  0.  patent, 

.  S.  closed. 

defect  V 

s. 

defect  V.  S. 

1 

0 

0 

1 

0 

2 

1 

1 

1 

3 

1 

2 

0 

0 

1 

0 

0 

4 

0 

3 

0 

0 

1 

0 

0 

1 

1 

11 


Many  patients  who  survive  until  early  adult  life  die,  not  of  the  lesion, 
but  of  pulmonary  tuberculosis.  The  frequency  of  this  disease  in  pul- 
monary stenosis  and  its  grave  prognosis  can  be  no  longer  disputed. 
The  cause  of  the  predisposition  seems  to  be:  (1)  The  reduced  blood 
supply  to  the  lungs  produces  an  anemic  condition  which  favors  infection; 
(2)  the  marked  cyanosis  usually  present  depresses  the  general  powers  of 
resistance  and  tends  to  destructive  tissue  metabolism;  (3)  the  subjects 
of  pulmonary  stenosis  frequently  live  to  an  age  when  tuberculosis  is 


398  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

likely  to  invade  the  organism  when  the  nutrition  is  low.  This  last  point 
is  illustrated  in  an  interesting  way  in  this  series.  Among  the  18  patients 
with  closed  septum,  in  whom  the  duration  of  life  was  longer,  pulmonary 
tuberculosis  occurred  7  times.  Among  the  remaining  64  patients  with 
defect  of  the  interventricular  septum  (in  whom  life  was  shorter)  it  occurred 
in  only  9  cases,  making  an  incidence  of  16  cases  in  the  82. 

Another  not  infrequent  termination  is  by  infection  from  an  acute 
endocarditis  developing  at  the  seat  of  the  defect.  Robinson  reports 
two  instances  in  patients  who  both  died  at  the  age  of  twenty  years,  in 
both  of  whom  the  conus  formed  a  separate  chamber  with  narrow  bulbar 
orifice.  In  the  one  case  there  were  large  vegetations  on  the  conus  wall, 
in  the  other  these  formed  a  fine  fringe  around  its  ventricular  orifice, 
and  coarse  outgrowths  about  the  associated  defect  in  the  interventricular 
septum.  Acute  endocarditis  appears  to  be  especially  common  in  this 
form  of  conus  deformity,  and  among  the  19  cases  in  which  the  conus 
formed  a  separate  chamber,  recent  vegetations  fringing  the  conus  orifice, 
on  the  wall,  or  on  the  tricuspid  valve  were  present  in  6.  In  Saundby's 
case  there  was  a  vegetative  arteritis  of  the  pulmonary  artery,  but  none 
of  the  valves. 

DILATATION  OF  THE  PULMONARY  ARTERY. 

Dilatation  of  the  pulmonary  artery  is  very  common  in  combination 
with  certain  cardiac  anomalies  but  is  rare  as  an  isolated  condition.  A 
few  cases  are  recorded  in  which  it  appears  to  be  primary  and  to  originate 
in  an  irregular  division  of  the  common  arterial  trunk.  The  main  vessel 
is  diffusely  enlarged  and  its  branches  are  tortuous  and  dilated,  but  the 
heart  and  lungs  are  otherwise  normal.  The  artery  is  usually  dilated  in 
persistence  of  the  fetal  passages  connecting  the  two  sides  of  the  heart, 
especially  in  patent  ductus  and  defects  of  the  lower  part  of  the  inter- 
auricular  septum,  or  in  widely  patent  foramen  ovale.  In  the  two  latter 
conditions  hypoplasia  of  the  aorta  is  frequently  associated  and  it  is 
difficult  to  say  which  is  the  primary  condition.  The  dilated  artery  may 
be  atheromatous  even  in  young  subjects. 

In  defects  of  the  interventricular  septum  at  the  base,  the  hypoplasia 
of  the  pulmonary  artery  so  often  present  may  give  place  to  a  marked 
dilatation.  This  was  the  case  in  9  among  the  34  primary  defects  of 
the  septum  in  this  series.  The  clinical  manifestations  of  pulmonary 
dilatation  are  discussed  by  Abrahams^  with  the  report  of  a  case  in  which 
this  was  diagnosed  as  the  primary  condition. 

CONGENITAL  AORTIC  STENOSIS  OR  ATRESIA. 

Subaortic  Stenosis. — This  term  has  been  applied  to  a  curious  annular 
thickening  of  the  endocardium  of  the  left  ventricle,  a  few  millimeters 
below  the  aortic  valves,  which  involves  the  base  of  the  aortic  segment  of 
the  mitral  valve,  and  encircles  the  ventricular  wall  at  this  point,  and 

1  Jour.  Am.  Med.  Assn.,  1913,  Ix,  1150. 


CONGENITAL  CARDIAC  DISEASE  399 

leads  in  most  of  the  cases,  to  a  localized  narrowing  of  the  cavity.  The 
cases  recorded  are  not  numerous  (seven  in  all),  but  the  condition,  when 
present,  usually  leads  to  serious  results,  and  is  therefore  important.  The 
thickened  ring  of  tissue  is  often  the  seat  of  a  chronic  inflammatory 
process,  probably  of  later  incidence,  but  there  can  be  little  doubt  that  it 
is  itself  of  congenital  origin.  Microscopic  examination  of  the  ring  in  a 
case  reported  by  Moore  showed  it  to  be  non-inflammatory  in  character. 
Keith  explains  it  as  an  arrest  of  development,  analogous  to  the  conus 
stenosis  of  the  right  ventricle,  the  bulbus  failing  to  atrophy  about  the 
root  of  the  aorta. 

Endocarditis  frequently  develops  both  at  the  defect  and  at  the  aortic 
valves  above  it,  and  may  lead  to  further  contraction  at  these  points. 
Shennan^  and  Smart^  report  two  such  cases  under  the  term  "double 
aortic  stenosis,"  and  a  third  is  recently  recorded  by  Thursfield  and  Scott.^ 
In  the  last,  the  aortic  orifice  was  narrowed  by  a  fibrous  ring,  situated 
on  the  interventricular  septum,  just  below  the  undefended  space  and 
extending  over  the  anterior  mitral  segment,  and  the  aortic  valves  were 
thickened  and  fused;  there  was  a  thin  line  of  fibrosis  in  the  otherwfse 
healthy  aorta  just  above  the  margin  of  the  valves,  and  slight  coarctation 
at  the  isthmus.  In  Shennan's  patient,  and  in  that  of  Fletcher  and 
Beattie,  a  thick  calcareous  ring  lay  below  the  thickened  and  ulcerated 
valves  which  were  the  seat  of  a  malignant  endocarditis. 

Most  of  these  patients  reach  adult  life,  and  the  clinical  significance  of 
the  condition  lies  chiefly  in  the  frequent  incidence  of  acute  endocarditis. 
The  picture  is  that  of  an  acquired  aortic  stenosis.  Slight  symptoms  may 
exist  from  childhood,  or  no  sign  of  the  presence  of  the  defect  may  be  given 
until  an  acute  endocarditis  develops,  or  failing  compensation  sets  in. 
The  systolic  murmur  produced  by  the  constriction  may  be  very  rough, 
and  audible  some  distance  from  the  chest  wall.  A  precordial  thrill 
existed  in  some  cases. 

Congenital  Stenosis  and  Atresia  of  the  Aortic  Orifice. — Aortic  stenosis 
of  antenatal  origin  is  not  common  and  the  duration  of  life  with  it  is 
very  short.  Two  forms  may  be  distinguished;  those  apparently  inflam- 
matory, with  the  stenosis  limited  to  the  valves  and  the  ventricular  septum 
entire,  and  those  apparently  due  to  an  arrest  in  development.  Unlike 
pulmonary  stenosis,  the  inflammatory  forms  are  here  the  commoner, 
fetal  endocarditis,  although  rare  in  the  left  heart,  usually  involving  the 
aortic  orifice.  Theremin  collected  17  cases,  in  only  2  of  which  was  there 
a  defect  of  the  septum.  In  our  series  there  are  6  cases,  5  of  which  are 
inflammatory. 

Monckeberg^  reports  a  case  of  inflammatory  origin  in  an  infant  of 
four  days,  and  refers  to  12  cases  in  the  literature.  He  ascribes  the 
occurrence  of  the  fetal  inflammation  to  probably  the  fifth  month  of 
gestation,  for  the  left  heart  was  very  small,  and  the  right  side  so  hyper- 
trophied  that  the  apex  of  the  right  ventricle  formed  a  recess-like  cavity 
below  the  left,  a  condition  typical  of  a  heart  in  which  the  right  ventricle 
had  carried  on  the  systemic  circulation  through  the  ductus  arteriosus 

1  Lancet,  1905,  vol.  i,  21.  ^  /f,^^,^  1904,  vol.  u,  1417. 

3  Brit.  Jour.  Child.  Dis.,  1913,  x,  104.  *  Deut.  path.  Gesell,  1907,  xi,'224. 


400  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

for  a  long  time.  In  aortic  atresia  the  left  ventricle  is  aplastic,  and  when 
a  defect  of  the  septum  exists,  may  even  be  obliterated.  The  foramen 
ovale  and  ductus,  are  nearly  always  widely  patent,  the  latter  supplying 
the  systemic  circulation. 

Cyanosis  is  usually  slight  or  absent  in  cases  of  aortic  stenosis,  but  marked 
in  atresia.  Physical  signs  may  be  absent,  or  there  may  be  a  loud  systolic 
or  double  murmur  heard  over  the  whole  chest.  In  both  conditions  the 
duration  of  life  is  very  short.  In  aortic  atresia  the  highest  age  attained 
was  twenty-seven  weeks.  Simmons^  described  an  interesting  case  in  an 
infant  aged  sixteen  weeks,  cyanotic  from  birth,  with  widely  patent  foramen 
ovale  and  ductus  arteriosus,  the  left  ventricle  aplasic,  the  right  ventricle 
greatly  hypertrophied,  and  the  aortic  cusps  fused  to  form  a  cone. 

Left-sided  Conus  Stenosis. — Schmincke^  describes  two  cases  in  adults 
of  a  peculiar  muscular  stenosis  of  the  conus  of  the  left  ventricle,  with 
healthy  aortic  valves,  and  no  apparent  cause,  which  he  thought  must 
be  of  congenital  origin,  due  to  a  primary  asymmetry  in  the  formation 
of  the  left  ventricle. 

ANOMALIES  OF  THE  SEMILUNAR  CUSPS. 

These  cusps  may  be  increased  or  diminished  in  number  and  defective, 
fenestrated,  or  otherwise  malformed.  A  row  of  supplementary  cusps 
may  exist  or  they  may  be  the  seat  of  attachment  of  anomalous  bands. 

Increase  in  Number. — Supernumerary  cusps  sometimes  occur  in  the 
pulmonary  artery  and,  less  frequently  in  the  aorta.  A  more  or  less  per- 
fectly formed  fourth  cusp  of  varying  size,  but  frequently  smaller  than 
normal,  may  be  inserted  between  two  of  the  others.  Or  the  usual  number 
of  segments  may  exist,  and  the  sinus  behind  one  of  these  be  divided  by  a 
raphe  which  runs  from  the  back  of  the  cusp  to  the  aorta,  indicating 
fusion  of  the  additional  segment  or  imperfect  division  from  its  fellows. 
In  rare  instances  five  cusps  occur.  Peacock  figures  a  case  of  five  aortic 
cusps,  and  Dilg  enumerates  from  the  literature  4  cases,  in  2  of  which  the 
five  cusps  were  in  the  aorta,  and  in  the  pulmonary  artery. 

The  supernumerary  cusps  have  sometimes  been  explained  as  an  effort 
at  repair  of  some  inflammatory  process  of  long  standing,  but  when  the 
fourth  segment  is  perfectly  formed,  or  the  raphe  indicating  it  shows  no 
sign  of  thickening  (as  in  a  case  in  the  McGill  Museum),  a  true  mal- 
formation must  be  concluded,  which  is  usually  explained  as  a  formation 
by  excess.  As  this  condition  is  of  congenital  origin,  the  cusps  are  generally 
so  adapted  to  each  other  as  to  be  competent  to  close  the  orifice,  no 
insufficiency  resulting;  they  occur  usually  in  a  heart  free  from  other 
malformations,  and  are  of  very  infrequent  occurrence.  Their  clinical 
significance  is  slight,  and  lies  chiefly  in  their  tendency,  like  all  valvular 
anomalies,  to  become  the  seat  of  endocarditis. 

Diminution  in  Number. — A  bicuspid  pulmonary  valve  is  not  uncommon 
with  other  cardiac  anomalies,  especially  transposition  and  septal  defects. 
A  bicuspid  aortic  valve  usually  occurs  in  an  otherwise  normal  heart  or 

1  Intercol.  Jour,  of  Austral.,  February  20,  1906. 

2  Deutsch.  med.  Wchnschr.,  1907,  xxxiii,  2082. 


CONGENITAL  CARDIAC  DISEASE  401 

associated  with  coarctation  or  hypoplasia  of  the  aorta.  In  some  instances 
both  pulmonary  and  aortic  valves  may  be  bicuspid.  The  anomalous 
segments  may  be  large,  with  smooth  surfaces,  showing  no  sign  of  further 
division,  a  true  reduction  in  number  existing;  or  one  or  both  may  present 
on  the  arterial  aspect  a  ridge  or  raphe  imperfectly  dividing  the  sinus 
behind  it  into  two  parts,  and  indicating  either  a  fusion  of  two  formerly 
independent  segments  or  a  beginning  separation  of  a  single  cusp  into 
two  elements ;  in  other  instances  a  single  membranous  ring  or  diaphragm 
may  exist  with  two  such  raphes  on  its  aortic  surface,  indicating  the  union 
with  each  other  of  all  three  cusps.  Where  such  a  raphe  is  absent,  the 
condition  is  undoubtedly  a  true  malformation,  but  where  this  exists, 
the  origin  of  the  bicuspid  state  of  the  valve  admits  of  much  discussion. 
Peacock  arrived  at  the  conclusion  that  the  majority  of  cases  were  con- 
genital, due  either  to  an  original  malformation  or  to  fusion  in  a  fetal 
endocarditis. 

A  series  of  cases  illustrating  the  same  line  of  thought  was  published  by 
Osier. ^    As  pointing  to  a  fusion  originating  in  fetal  life,  he  enumerates 

(1)  the  presence  of  a  low,  sometimes  half  obliterated,  raphe  behind  one 
of  the  cusps;  (2)  compensatory  changes  in  the  fused  cusps,  so  that  their 
free  edge  becomes  equal  to  or  even  shorter  than  the  single  segment;  and 
(3)  the  fusion  of  the  coronary  or  right  and  left  segments  of  the  valve. 
That  the  lateral  (right  and  left)  segments  are  the  seat  of  the  congenital 
fusion,  the  posterior  cusp  remaining  always  single,  is  stated  also  by 
B  ir  ch-Hir  s  chf  eld . 

Thickening  of  cusp  and  raphe  does  not  prove  that  the  fusion  is  not  of 
"congenital  origin,  for  endocarditis  is  likely  to  supervene  on  any  valvular 
anomaly.  On  the  other  hand,  it  is  admitted  that  many  cases  are  of  post- 
natal inflammatory  origin.  Such  postnatal  fusion  may  be  reasonably 
deduced  when  (1)  the  two  sections  of  the  cusp  are  approximately  equal; 

(2)  when  the  raphe  dividing  them  has  its  superior  origin  on  a  level 
with  the  superior  origin  of  the  unaffected  cusp  (instead  of  at  the  lower 
level,  as  described  by  Osier);  and  (3)  when  in  addition  the  only  thick- 
ening observable  is  in  the  angle  betiveen  the  fused  cusys.  This  latter  point 
has  been  called  to  the  attention  of  the  T\Titer  by  Dr.  Adami,  who  points 
out  that  the  free  part  of  the  cusps,  being  in  constant  motion,  is  but  little 
liable  to  undergo  inflammatory  adhesion,  and  that  this  will  be  prone  to 
occur  at  the  point  of  their  insertion  into  the  orifice  wall,  which  is  the 
only  portion  of  their  edge  that  is  fixed  and  relatively  motionless.  There- 
fore, inflammation  originating  at  the  angle  of  junction  of  contiguous 
cusps  may  lead  to  localized  obliteration  of  the  prinaary  angle,  or  to  fusion 
of  the  cusps  with  a  new  immovable  angle  of  junction  further  removed 
from  the  aortic  wall,  a  progressive  fusion  occurring. 

When  a  thin  delicate  raphe  exists  behind  one  of  the  segments  of  a 
bicuspid  valve  vdthout  any  trace  of  thickening.  Babes  insists  that  it 
cannot  be  ascribed  to  a  fusion  in  fetal  endocarditis,  but  must  be  regarded 
as  a  true  malformation  dependent  perhaps  on  incomplete  division  of  an 
originally  single  cusp.    The  semilunar  cusps  originate  from  the  four  distal 

1  Montreal  General  Hasp.  Reports,  1880. 
YOL.   IV — 26 


402  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

bulbar  endocardial  ridges,  two  of  which  are  divided  by  the  descent  of  the 
aortic  septum,  so  that  a  third  segment  is  placed  in  each  artery.  The  four 
cusps  of  the  undivided  truncus  are  sometimes  seen  in  the  cases  of 
common  arterial  trunk. 

The  results  upon  the  heart  and  circulation  of  a  reduction  in  the  number 
of  the  aortic  cusps  maj'-  be  summed  up  as  follows: 

1.  The  segments  may  approximate  and  be  perfectly  competent,  no 
pathological  effects  ensuing.  This  is  proved  by  the  occasional  finding 
of  a  bicuspid  valve  in  healthy  adults  even  of  advanced  age. 

2.  The  gradual  bulging  of  the  cusps,  their  greater  length,  the  free 
space  that  sometimes  exists  between  them,  perhaps  a  yielding  of  the 
aortic  ring,  lead  frequently  to  a  valvular  insufficiency  or  to  a  narrowing 
of  the  orifice. 

3.  Endocarditis  commonly  supervenes,  either  as  the  acute,  often 
malignant  form,  or  as  a  chronic  inflammatory  process,  leading  to  thicken- 
ing and  deformity  of  the  cusps  and  to  subsequent  valvular  disease. 

4.  Atheromatous  changes  at  the  base  of  the  aorta  have  been  frequently 
noted,  and  in  6  of  the  11  cases  described  by  Babes  and  Deteindre  there 
was  an  aneurismal  bulging  of  the  right  posterior  wall  of  the  aorta,  which 
formed  in  5  instances  a  definite  aneurism,  from  the  rupture  of  which, 
in  2  cases,  death  ensued.  This  is  seen  also  in  two  cases  in  the  McGill 
Museum. 

This  remarkable  combination  of  an  aneurism  of  the  base  of  the  aorta 
with  a  bicuspid  valve  is  believed  by  Babes  to  be  directly  connected  with 
the  bicuspid  character  of  the  aortic  cusp  below  it,  and  is  ascribed  by 
him  (a)  to  an  extension  of  the  same  thinning  or  trophic  process  that  led 
to  the  anomalous  condition  of  the  cusp,  (b)  to  the  lack  of  support  given 
to  the  aorta  at  this  its  weakest  point,  and  (c)  to  the  frequent  insufficiency 
of  the  cusps  and  the  yielding  of  the  aortic  ring. 

Miscellaneous  Anomalies. — Dilg  reports  a  remarkable  case,  in  a  child 
aged  two  years,  of  an  endocardial  fold  divided  roughly  into  two  cusps 
with  their  convexity  toward  the  ventricle,  just  below  the  base  of  a  bicuspid 
aortic  valve,  both  coronaries  being  behind  one  cusp.  Banks^  reported 
a  woman,  aged  thirty-four  years,  with  physical  signs  of  aortic  insufficiency 
and  a  loud,  musical  murmur  at  the  base,  audible  at  some  distance  from 
the  chest,  whose  heart  was  hypertrophied  and  presented  a  cribriform 
condition  of  the  aortic  valve,  and  one-quarter  inch  below  it  in  the  left 
ventricle  three  other  rudimentary  cusps.  These  may  be  of  compensatory, 
postnatal  origin,  as  in  a  number  of  other  cases  recorded  of  long-standing 
aortic  insufficiency. 

In  a  case  in  the  McGill  Museum,  reported  by  Campbell  and  Hepburn,^ 
of  pulmonary  conus  stenosis  with  septal  defect,  two  well-formed  valves 
are  situated  1.8  cm.  behind  the  pulmonary  semilunar  cusps,  at  the  point 
which  marks  the  lower  bulbar  orifice,  and  the  site  of  the  proximal  bulbar 
swelling  in  the  embryo.  This  is  an  entirely  unique  finding  and  suggests 
an  atavistic  reversion  to  the  cusps  seen  at  this  point  in  the  dipnoan  heart. 

In  one  of  Babes'  cases  of  bicuspid  aortic  valves,  a  peculiar  band,  like 

1  Dublin  Hosp.  Gazette,  1857,  p.  330. 

2  Can.  Med.  Assn.  Jour.,  1913,  iii,  871. 


CONGENITAL  CARDIAC  DISEASE  403 

a  papillary  muscle  of  the  mitral  valve,  traversed  the  sinus  of  Valsalva. 
Hektoen^  quotes  from  the  literature  several  other  instances  of  anomalous 
cords  at  the  level  of  the  valves,  and  a  case  observed  by  himself  of  a  large 
defect  at  the  base  of  one  of  the  segments,  all  of  which  he  ascribed  to 
defects  in  the  development  of  the  aortic  septum. 

Defective  Development  of  the  Semilunar  Cusps. — In  a  few  instances 
of  bicuspid  valve  a  gap  may  be  left  on  the  wall  of  the  vessel  between 
the  segments  where  evidently  no  third  cusp  has  formed.  This  occurred 
in  two  of  Deteindre's  series.  A  remarkable  instance  of  such  a  defect 
in  the  pulmonary  valve  is  recorded  by  Stinzing.  Here  there  are  only 
two  pulmonary  cusps,  and  a  large  free  space  occupying  the  position 
of  the  third  was  traversed  by  two  low  ridges,  evidently  its  rudiments. 
The  heart  was  from  a  woman,  aged  sixty-four  years,  presenting  signs 
and  symptoms  of  pulmonary  insufficiency,  a  history  of  pneumonia 
eight  months  before  death,  and  failing  compensation  since. 

PRIMARY  DEFECTS  OF    THE    AURICULOVENTRICULAR    ORIFICES. 

Congenital  disease  of  the  auriculoventricular  valves  differs  from  that 
of  postnatal  life  chiefly  in  its  infrequency,  in  the  more  extreme  character 
of  the  process,  atresia  being  more  common  than  stenosis,  and  in  the  fact 
that  the  right  side  of  the  heart  is  usually  affected  rather  than  the  left. 
Owing  to  the  rarity  of  the  cases,  to  the  short  duration  of  life,  and  to  the 
fact  that  in  the  infant  heart  the  picture  presented  is  hard  to  distinguish 
from  that  of  the  more  frequent  lesions  at  the  arterial  ostia,  this  subject 
is  not  of  great  clinical  importance,  and  its  chief  interest  lies  in  the  contri- 
bution which  it  brings  to  our  information  upon  the  question  of  the  patho- 
genesis of  cardiac  defects. 

Tricuspid  Stenosis. — Although  this  lesion  is  not  very  uncommon 
in  adults  the  cases  which  can  be  proved  to  have  originated  in  intrauterine 
life  are  very  rare.  Vierordt  knew  of  only  three  instances,  unassociated  with 
disease  of  the  pulmonary  valves,  in  the  literature.  In  combination  with 
pulmonary  stenosis  or  atresia  it  is  more  frequent.  A  good  illustration 
of  the  latter  combination  is  seen  in  a  specimen  in  the  McGill  Museum,  pre- 
sented by  Sir  William  Osier.  In  the  heart  of  a  cyanotic  infant  aged  four 
months,  both  pulmonary  and  tricuspid  valves  are  thickened,  shortened, 
and  fused,  and  their  orifices  markedly  reduced;  the  ventricular  septum 
is  entire,  the  foramen  ovale  widely  patent,  the  right  auricle  hugely  dilated, 
and  the  tricuspid  surmounted  by  recent  vegetations.  Such  cases  are 
undoubtedly  of  inflammatory  origin,  and  are  of  value  as  proving  that 
fetal  endocarditis,  although  it  has  been  overrated  as. a  cause,  certainly 
has  its  place  as  an  etiological  factor  in  congenital  cardiac  disease. 

Tricuspid  Atresia. — Although  in  itself  rare,  tricuspid  atresia  is  the 
commonest  of  all  congenital  lesions  of  the  auriculoventricular  cusps. 
Rauchfuss  collected  16  cases  from  the  literature  of  which  5  were  due  to  a 
defect  in  development,  5  were  apparently  inflammatory,  and  the  re- 
mainder were  of  "doubtful"  origin.     Since  then  additional  cases  have 

1  Chicago  Path.  Soc,  1905. 


404  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

been  reported  by  Chapotot  (quoted  by  Vierordt),  Sieveking,  Kiihiie^ 
(two  cases)  Bernstein^  and  Wieland.^ 

Pathological  Anatomy  and  Pathogenesis. — Cases  of  inflammaton'  origin 
must  be  distinguished  from  those  due  to  a  defect  in  development,  for  the 
latter  present  several  points  of  special  interest.  Those  of  inflammatory 
origin  have  usually  progressed  tlirough  a  stenosis  and  show  distinct 
evidence  of  an  antenatal  valvuhtis  in  the  form  of  an  extensive  cicatricial 
contraction  of  the  endocardium  adjacent  to  the  obliterated  tricuspid 
orifice,  and  often  of  the  pulmonary  valves.  The  developmental  cases,  on 
the  other  hand,  may  present  no  sign  of  inflammation,  but  the  tricuspid 
orifice  is  absent,  and  either  shows  no  trace  of  its  presence,  or  this  is 
marked  by  a  shallow  groove,  the  tricuspid  segments  are  lacking,  and  the 
right  auricle  is  divided  from  the  right  ventricle  by  a  thick  muscular 
septum.  Kiihne,  and  subsequently  Wieland,  subdivided  these  develop- 
mental cases  into  a  group  of  (a)  "isolated"  primary  atresias  in  which 
certain  pathological  changes  of  a  secondary  nature  are  constant  so  that  a 
definite  type  is  set  up,  and  (6)  tricuspid  atresia  complicated  by  other 
grave  cardiac  anomalies  of  independent  origin,  such  as  transposition 
of  the  arterial  trunks,  pulmonary  atresia,  etc.  Eight  cases  of  the  "iso- 
lated" form  that  constitutes  the  first  group  were  separated  by  Kiihne, 
and  later  by  Wieland,  from  the  others.  To  these  may  be  added  one 
by  Bernstein  from  our  series.  The  changes  in  all  of  these  are  practically 
identical  and  clearly  indicate  the  sequence  of  events.  There  is  an 
entire  absence  of  the  tricuspid  orifice,  and  the  body  of  the  right  ventricle  is 
an  aplastic  structiu-e,  while  the  left  is  highly  hypertrophied  and  dilated, 
appearing  at  first  sight  to  form  the  whole  heart,  with  the  right  chamber 
as  an  appendage  to  it;  the  right  auricle  is  also  hugely  dilated  and  the 
foramen  ovale  is  widely  patent,  or  a  defect  of  the  interventricular  septum 
exists;  in  addition  there  is  always  a  defect  in  the  muscular  interventricular 
septum  leading  from  the  cavity  of  the  left  ventricle  into  the  dilated  conus 
of  the  right  ventricle  and  thence  into  the  pulmonary  artery.  The  course 
of  the  circulation  is  necessarily  from  right  to  left  auricle  through  the  fora- 
men ovale,  and  thence  to  the  left  ventricle,  from  which  the  blood  is 
distributed  in  part  to  the  aorta  and  in  part  through  the  septal  defect  to 
the  pulmonary  artery.  The  aorta  is  usually  dilated,  and  the  pulmonary 
is  normal  or  somewhat  reduced  in  size  owing  to  the  smallness  of  the 
chamber  from  which  it  springs. 

These  cases  of  "isolated  primary  tricuspid  atresia"  are  of  interest 
from  the  standpoint  of  the  pathogenesis  of  ventricular  septal  defects, 
for  they  show  that  the  old  mechanical  or  congestive  theory  of  septal 
defects  in  pulmonary  stenosis,  in  which  the  defect  was  thought  to  be 
secondary  to  the  raised  pressure  in  the  right  ventricle,  which  has  long 
been  justly  discarded  in  this  connection,  at  least  in  regard  to  the  develop- 
mental forms  of  pulmonary  stenosis,  must  be  accredited  here.  That  is 
to  say,  the  septal  defect  in  tricuspid  atresia  is  here  evidently  secondary 
to  the  congestion  in  the  left  ventricle  which  forces  an  outlet  in  the  conus 

1  Jahrb.  f.  Kinderh.,  1906,  Ixiii,  235. 

-  New  York  Path.  Soc.  Reports,  February,  1906. 

3  Jahrb.  f.  Kinderh.,  1914,  Ixxix,  .320. 


CONGENITAL  CARDIAC  DISEASE  405 

of  the  pulmonary  artery  and  this  allows  the  circulation  to  be  maintained. 
Wieland  points  out  and  insists  on  the  importance  of  Kiihne's  separation 
of  this  group,  with  its  constant  secondary  complex,  on  this  account. 

It  should  be  remembered  that  cases  of  developmental  tricuspid  atresia 
with  or  without  complicating  cardiac  anomalie,  constitute  one  well 
recognized  form  of  the  cor  biatriatrum  triloculare.  The  subject  is  dis- 
cussed under  that  head. 

Etiology. — The  causation  of  the  cases  of  inflammatory  origin  is  that 
of  fetal  endocarditis  elsewhere.  In  tricuspid  atresia  the  secondary 
ventricular  septal  defect  is  bound  to  occur,  for  the  proper  maintenance 
of  the  circulation  as  well  here  as  in  the  developmental  forms. 

Much  interest  attaches  to  the  causation  of  the  developmental  forms 
of  tricuspid  atresia.  In  the  embryo  the  auricular  canal  opens  at  first 
by  a  common  orifice  into  the  left  side  of  the  common  ventricle,  and  later 
by  a  process  of  shifting  to  the  right  comes  to  lie  more  in  the  median  line. 
The  theory  had  been  advanced  that  either  a  lack  or  an  exaggeration  of 
this  shifting  to  the  right  would  lead  to  a  wrong  adjustment  of  the  parts, 
and  to  a  mitral  or  tricuspid  atresia.  Again,  the  auriculo ventricular 
orifice  is  divided  into  the  mitral  and  tricuspid  ostia  b}^  the  growth  of 
endocardial  cushions  in  its  centre,  and  by  a  union  of  these  with  the  inter- 
auricular  and  interventricular  septa.  Should  these  cushions  become 
deviated  to  the  right  or  to  the  left  in  their  formation  they  may  become 
adherent  to  the  corresponding  wall  of  the  common  ostium  and  thus  lead 
to  tricuspid  or  mitral  atresia.  Rokitansky  thus  explains  his  case  of 
mitral  atresia.  In  that  by  Robertson  the  tricuspid  atresia  present  was 
ascribed  to  a  fusion  of  the  endocardial  cushions  with  the  malposed 
auricular  secondary  septum,  in  consequence  of  a  persistence  of  the 
valvulae  venosse  which  formed  a  coarse  network  across  the  cavity  of 
the  right  auricle.  The  suggestion  has  also  been  made  that  a  premature 
obliteration  of  the  ductus  arteriosus  during  early  fetal  life,  might  lead 
to  aplasia  of  the  right  ventricle  and  tricuspid  orifice  by  cutting  this 
part  of  the  heart  out  of  the  fetal  circulation.  This  possibility  is  disproved, 
however,  by  the  fact  that  in  several  of  the  cases  this  passage  is  freely 
patent.  The  conclusion  remains  that  the  probable  causation  of  mitral 
and  tricuspid  atresia  lies  in  the  mal-position  and  irregular  union  of 
those  parts  of  the  cardiac  septa  dividing  the  mitral  from  the  tricuspid 
ostium. 

Symptoms  and  Signs  of  Tricuspid  Stenosis  not  Atresia. — Cyanosis  may 
be  present  from  birth,  or  may  develop  after  a  few  days  or  weeks.  In  the 
classical  developmental  type  described  above  it  is  usually  extreme  in  the 
end,  though  its  onset  may  be  delayed  for  some  time.  In  Bernstein's 
case,  aged  two  years  and  eight  months,  it  did  not  appear  until  the  six- 
teenth month,  but  then  became  marked  with  clubbing,  and  a  polycy- 
themia of  10,000,000  developed.  This  late  appearance  was  possibly 
explained  by  the  absence  in  this  case  of  the  auricular  septum,  a  condition 
which  must  have  facilitated  the  circulation.  On  the  other  hand,  Ivelley's 
patient,  a  delicate,  unhealthy  child,  showed  only  slight  lividity  on  crying 
or  wdien  he  had  a  cold,  and  in  Sieveking's  case,  dying  at  nine  weeks, 
cyanosis  was  absent  throughout,  but  dyspnoea  was  a  prominent  symptom. 


406  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

Dyspnceic  attacks  are  a  prominent  feature,  are  often  of  daily  occurrence 
and  are  frequently  the  direct  cause  of  death. 

Physical  signs  are  not  very  characteristic,  being  obscured  by  those  of 
the  septal  defect  that  in  tricuspid  atresia  is  always  present,  and  by  the 
fact  that  a  systolic  murmur  with  maximum  intensity  over  the  right 
ventricle,  such  as  is  usually  produced  in  these  cases,  may  with  difficulty, 
be  distinguished  from  one  generated  at  the  pulmonary  area.  The  marked 
hypertrophy  of  the  left  combined  with  the  smallness  of  the  right  ventricle 
is  of  assistance  in  the  differential  diagnosis  from  pulmonary  valve 
disease,  although  allowance  must  be  made  for  the  increased  cardiac  area 
produced  by  dilatation  of  the  right  auricle.  This  feature  was  indicated 
in  Wieland's  patient  by  a  zone  of  dulness  to  the  right  of  the  vertebral 
column  behind.  His  case  was  characterized  also  by  a  strong  systolic 
murmur  and  precordial  thrill  of  maximum  intensity  at  the  apex,  both 
of  which  were  of  a  curiously  intermittent  character. 

Duration  of  Life. — -Very  few  of  the  developmental  cases  of  tricuspid 
atresia  live  more  than  one  year.  Bernstein's  patient  reached  two  years 
and  eight  months,  a  relatively  high  age  that  is  perhaps  explained  by  the 
almost  complete  absence  of  the  interauricular  septum  that  was  present. 
In  the  inflammatory  cases,  in  which  the  atresia  has  probably  progressed 
through  a  stenosis,  adult  life  is  frequently  attained.  Such  was  Bierdach's 
case  dying  at  twenty-eight  years. 

Congenital  Mitral  Stenosis. — This  is  even  rarer  than  the  same  lesion 
at  the  tricuspid  orifice.  A  typical  case  evidently  due  to  an  antenatal 
valvulitis,  in  a  child  of  ten  months,  with  cyanosis  and  dyspnoea  from 
birth,  is  reported  by  Simmons.  A  curious  combination  of  dwarfism 
and  mitral  stenosis  in  patients  who  have  attained  adult  life  has  been 
observed.  L'Abbe  reports  a  case  in  a  woman,  aged  twenty-seven 
years,  of  extremely  small  stature  (1  meter  high,  43  kilos  weight),  puerile 
intelligence,  and  marked  infantilism.  There  was  a  clear  history  as 
well  as  physical  evidence  of  congenital  syphilis,  and  a  pure  mitral 
stenosis. 

Mitral  Atresia. — A  complete  obliteration  of  the  mitral  orifice  is  still 
rarer  than  stenosis.  The  same  remarks  apply  in  regard  to  etiology  as 
in  tricuspid  atresia,  but  here  a  primarj'^  defect  in  development  may  be 
almost  constantly  assumed.  Grave  associated  anomalies  are  also  nearly 
always  present,  and  give  additional  proof  of  a  teratological  origin.  In 
Theremin's  observation  of  an  infant  aged  two  days,  the  left  auricle 
and  ventricle  were  aplastic  without  any  trace  in  the  latter  of  a  mitral 
orifice,  its  walls  being  formed  throughout  of  finely  reticulated  muscle 
fibres;  the  foramen  ovale  was  closed,  the  interventricular  septum  defective, 
the  pulmonary  valve  bicuspid,  and  the  aorta  appeared  to  arise  from  the 
right  ventricle;  there  was  a  horseshoe  kidney  and  double  ureter.  Lawrence 
and  Nabarro  give  a  similar  case  of  mitral  atresia,  defect  of  the  septum, 
aplastic  left  ventricle,  the  aorta  arising  behind  the  pulmonary  artery 
from  the  right  ventricle,  with  coarctation  of  the  aorta,  transposition  of 
the  stomach,  absence  of  spleen  and  hepatic  section  of  inferior  vena  cava; 
anomalies  in  form  of  liver  and  lungs.  In  Rokitansky's  case  of  aortic 
and  mitral  atresia  with  defect  of  the  septum,  patent  foramen  ovale, 


CONGENITAL  CARDIAC  DISEASE  407 

and  dilated  pulmonary  artery,  in  a  child  aged  twelve  days,  there  was 
an  accessory  right  bronchus. 

Congenital  Mitral  and  Tricuspid  Insufficiency. — These  lesions  may 
result  from  a  primary  malformation  of  the  cusps  or  from  secondary 
deformity  in  the  arrest  of  development  of  neighboring  structures,  as 
in  persistent  ostium  primum.  Or  they  may  be  due  to  thickening  and 
shortening  of  the  valve  in  a  fetal  endocarditis;  thus  Barth  and  Roger 
describe  a  case  in  which,  on  auscultation  before  birth,  a  long,  loud,  rough 
murmur  was  heard  accompanying  the  heart  sounds.  The  child  was 
stillborn  three  days  later,  and  the  right  ventricle  was  found  dilated, 
the  tricuspid  orifice  enlarged,  and  its  cusps  shrunken  and  insufficient, 
and  evidently  the  seat  of  an  endocarditis.  In  the  case  of  Steffen,  of  a 
child  aged  ten  and  a  half  months,  there  were  no  tricuspid  segments, 
but  the  valve  formed  a  low  ridge  which  was  thickened,  reddened,  and 
slightly  jagged.  The  mitral  cusps  were  similarly  thickened  and  reddened, 
and  one  of  them  was  reduced  likewise  to  a  narrow  ridge. 

Congenital  Mitral  Insufficiency. — Steffen's  case  is  the  only  instance 
of  congenital  mitral  insufiiciency  found  in  the  literature.  True  congenital 
tricuspid  insufiiciency  is  also  rare,  probably  there  are  not  a  dozen  cases. 

ANOMALIES  OF  THE  AURICULOVENTRICULAR  CUSPS. 

Double  Auriculoventricular  Orifice.  —  A  second  valvular  opening 
supplied  with  its  own  cusps,  chordae  tendinese  and  papillary  muscles, 
may  lie  within  the  segments  of  an  otherwise  normal  auriculoventricular 
valve.  Seven  such  cases  are  recorded,  six  of  double  mitral  orifice  by 
Greenfield,^  Cohn,^  Degen,^  Stuhlenweisenberg,*  and  Camisa,^  and  one 
of  double  tricuspid  by  Pisenti.*'  In  Stuhlenweisenberg's  case,  and  in 
one  of  Camisa's,  the  two  orifices  were  of  equal  size,  and  were  separated 
by  a  bridge  of  valve  tissue  which  supplied  a  cusp  to  either  opening;  in 
all  the  other  cases  the  second  opening  was  much  smaller,  and  lay  in  one 
of  the  segments  of  the  primary  orifice. 

Two  hitherto  unpublished  cases  of  the  latter  description,  are  in  the 
collections  of  the  Harvard  and  the  McGill  Medical  Museums.  In  the 
Harvard  case,  an  opening  2  cm.  long  lies  in  the  aortic  cusp  of  the  main 
mitral  orifice,  and  leads  into  an  aneurismal  pouch  formed  by  the  apex 
of  this  segment,  which  communicates  with  the  cavity  of  the  ventricle 
by  numerous  fenestrations.  The  McGill  heart  (Fig.  37)  is  of  bizarre 
external  form  owing  to  its  bifid  apex,  deep  auriculoventricular  groove, 
and  hypertrophied  right  chambers.  The  inter  auricular  septum  presents 
a  small  valvular  patent  foramen  ovale  above,  and  is  absent  in  its  lower 
two-thirds,  a  large  crescentic  defect  {persisient  ostium  jprimum)  existing. 
The  mitral  valve  is  replaced  by  a  single  large  segment  which  is  cleft 
in  its  anterior  portion,  passing  forward  from  either  side  to  be  inserted 

1  Trans.  Path.  Soc,  1876,  xxvii,  128  (with  plate). 

2  Inaug.  Dissertation,  Konigsberg,  1896  (with  plate). 

3  Inaug.  Dissertation,  Greifswald,  1903. 

4  Centralb.  f.  Pathol,  1912,  xxiii,  1027.  _  ^  /^^(^^  p.  342 
^  Di  una  rarissima  Anomalia  della  tricuspide,  Perugia,  1888. 


408 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


into  the  middle  of  the  base  of  the  interventricular  septum  where  this 
bounds  the  interauricular  defect  below.  The  secondary  mitral  ostium 
lies  in  the  posterior  half  of  this  large  primary  segment,  7  mm.  back  from 
its  free  margin.  It  is  a  perfect  valvular  opening  admitting  a  lead-pencil 
with  two  well-formed  cusps  attached  to  slender  chordae,  arising  from_  two 
short  papillary  muscles  which  lie  behind  and  independent  of  the  single 
group  from  which  the  chordae  of  the  primary  segment  spring.  The  right 
auriculo ventricular  valve  is  malformed  and  an  irregular  excavation  in 
its  septal  cusp  suggests  an  unsuccessful  attempt  at  a  double  tricuspid 
ostium.     The  aortic  valve  is  bicuspid.     The  endocardium  is  healthy. 


Fig.  37 


Heart  of  a  child,  aged  five  years,  showing;  A,  defect  of  lower  part  of  interauricular  septum;  B,  patent 
foramen  ovale;  C,  double  mitral  orifice;  D,  cleavage  of  mitral  segment.  (From  a  specimen  in  the  Patho- 
logical Museum,  MoGill  University.) 


Etiology. — Camisa  believed  a  fetal  endocarditis  had  led  to  a  fusion 
of  segments  at  their  apices  and  to  the  formation  of  secondary  orifices. 
Cohn  and  Stuhlenweisenberg  suggest  a  malformation  by  excess,  a  view 
supported  in  the  latter's  case  by  the  equal  size  of  the  two  orifices.  Pisenti 
supposed  a  fenestration  of  the  endocardial  cushions,  which  had  trans- 
mitted the  blood  stream  in  early  embryonic  life  and  had  become  trans- 
formed into  a  second  valvular  orifice  by  a  natural  adaptation  of  growth, 
the  papillary  muscles  and  chordae  growing  up  to  its  borders. 

In  the  two  specimens  seen  by  us,  Pisenti's  explanation  seems  to 
apply,  the  marked  irregularities  in  both  auriculoventricular  valves  due 
to  the  auricular  septal  defect  in  the  McGill  specunen,  and  the  multiple 
fenestrations  in  the  Harvard  case,  alike  arguing  for  such  accidental 
origin  at  an  early  embryonic  period.  Camisa's  theory  of  a  fetal  endo- 
carditis is  not  tenable  in  the  case  of  the  McGill  specimen  and  others  in 
which  the  endocardium  is  free  from  every  trace  of  sclerotic  change. 


CONGENITAL  CARDIAC  DISEASE  409 

Symptoms. — The  double  orifice  is  in  itself  of  no  clinical  significance, 
the  secondary  segments  functioning  as  normal  valves.  In  the  majority 
of  the  cases,  including  Cohn's  patient,  who  died  at  seventy-one  years, 
both  sets  of  valves  were  thin,  healthy  and  competent.  Chronic  endo- 
carditis had  supervened  in  both  Camisa's  cases  and  in  Stuhlenweisen- 
berg's.  In  the  latter  a  loud  systolic  murmur  over  the  precordium  was 
associated  with  insufficiency  and  sclerosis  of  the  segments  of  the  anterior 
mitral  ostium,  the  posterior  remaining  free. 

Displaced  Orifice.— A  double  mitral  orifice  is  described  by  Andrewes,^ 
in  which  two  orifices  separated  by  a  fibrous  septum  lay  one  behind  the 
other  in  the  left  ventricle.  The  right  ventricle  was  rudimentarj'^,  the 
interventricular  septum  defective  and  the  tricuspid  valve  absent.  A 
deflection  of  the  septum  to  the  right  so  that  both  orifices  are  placed 
in  the  same  ventricle  was  assumed. 

Miscellaneous  Anomalies. — Various  minor  defects,  as  irregularly  formed 
or  accessory  leaflets  and  anomalous  arrangement  of  the  chordae  tendinese 
or  papillary  muscles  occasionally  occur,  and  may  in  some  instances 
contribute  to  an  insufficiency  of  the  valves. 

PRIMARY   PATENCY   AND    ANOMALIES    OF    THE    DUCTUS 
ARTERIOSUS    BOTALLI. 

The  ductus  arteriosus  of  the  fetus  is  a  short,  thick  trunk,  10  to  15  mm. 
long,  running  from  the  left  branch  of  the  pulmonary  artery  directly  after 
the  bifurcation  to  the  under  side  of  the  arch  of  the  aorta  just  beyond  the 
origin  of  the  left  subclavian  artery,  which  serves  to  carry  the  unaerated 
blood,  returned  from  the  head  and  upper  extremities,  to  the  descending 
aorta,  whence  it  passes  to  the  placenta.  At  birth  the  ductus  undergoes 
a  rapid  involution,  its  lumen  becomes  practically  impermeable  about 
the  third  week  of  life,  the  alterations  in  its  wall,  which  lead  to  its  per- 
manent obliteration,  going  on  for  some  months,  and  finally  transforming 
it  into  the  ligamentum^rt^riosum  of  Jater  life.  The  average  diameter 
of  the  patent  ductus  at  birth  is  given  by^VTerordt  at  5  to  6.8  mm.  and 
by  Theremin  as  4.8  mm.  But  when  filled  with  fluid  during  life,  or 
experimentally  injected  directly  after  death,  it  is  found  to  be  much 
larger.  Thus  in  a  series  of  infant  hearts  prepared  by  Klotz,  in  which 
he  injected  the  ductus  from  the  aorta  with  gelatin  at  autopsy,  it  was 
found  in  the  newly  born  to  be  fully  equal  in  size  to  the  main  pulmonary 
trunk.  He  ascribes  its  apparent  smallness  as  usually  seen  postmortem 
to  the  firm  contraction  of  the  muscular  wall. 

The  ductus  may  (1)  remain  patent  throughout  life,  (2)  undergo 
aneurismal  dilatation,  (3)  it  may  be  absent,  or  (4)  it  may  have  an 
anomalous  origin  or  course. 

Patency  of  the  ductus  is  not  infrequent  in  combination  with  other 
cardiac  defects,  especially  those  in  which  there  is  some  serious  inter- 
ference with  the  pulmonary  circulation.  It  occurred  in  166  of  this 
series  of  cases,  in  21  of  which  it  was  combined  with  pulmonary  atresia, 

^  Trans.  Path.  Soc,  London,  1903,  liv. 


410  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

in  14  with  pulmonary  stenosis,  and  in  23  with  transposition  of  the  great 
trunks.  As  an  isolated  condition  it  is  among  the  more  infrequent  of 
cardiac  anomalies.  The  first  carefully  recorded  case  of  primary  patency 
with  autopsy  findings,  was  diagnosed  before  death  and  published  by 
Bernutz^  in  1849.  Six  cases  were  collected  by  Almagro^  in  1862,  12  by 
Gerhardt^  in  1867,  20  by  Wrany^  in  1871,  and  26  by  Vierordt  in  1898. 
Herxheimer  enumerated  all  the  above  in  38  cases  collected  in  1910,  while 
Wells,^  in  1908,  found  41.  A  careful  anal}' sis  of  34  cases  with,  and  37 
without,  autopsy  report  was  published  by  Goodman^  in  1910,  and  there 
are  important  clinical  studies  by  Hochsinger,  Gillett,''  Taylor,^  and 
Wessler.^  From  these  and  other  sources,  64  cases  of  uncomplicated 
primary  patency  with  clinical  history  and  autopsy  reports,  have  been 
analyzed  in  this  series.  Of  these,  18  are  in  infants  under  two  years,  and 
46  in  "adults"  over  this  age.  As  of  special  interest,  or  not  included  by 
other  writers,  may  be  mentioned  the  cases  by  Hewitt,^*^  Hall,^^  Kingsley^^ 
Thompson^^  and  Carpenter^*  in  infants;  and  Kaulich,^^  Fagge,^^  Darier,^'^ 
Schrotter,^^  Drasche,^^  Garipuyj^"  Crouzet,^^  Greenhow,"^  Gibson,^^ 
Wells,  Schnitzler,  and  Mead,-^  in  adults,  as  well  as  13  adult  cases  quoted 
below,  in  which  the  patency  was  complicated  by  acute  infective  pul- 
monary endarteritis. 

Pathogenesis. — The  causes  of  persistent  patency  of  the  duct  are  to 
be  sought  in  the  conditions  of  its  normal  closure,  and  this  must  depend 
upon  the  influences,  mechanical  or  otherwise,  of  the  changes  in  the 
circulation  at  birth,  and  upon  the  consequent  alterations  in  the  vessel 
wall,  itself  a  fetal  structure  destined  to  involution.  As  possible  factors 
in  the  process  of  closure  may  be  enumerated:  (1)  peculiarities  in  the 
histological  structure  of  the  ductus  wall,  (2)  alterations  in  the  blood- 
pressure  at  birth,  (3)  modifications  at  birth  in  the  position  of  the  ductus 
relative  to  the  aorta  and  pulmonary  artery,  and  other  mechanical 
factors  preventing  entrance  of  blood  from  the  aortic  side. 

1.  Histology. — The  ductus  wall  is  poor  in  elastic  tissue  as  compared 
with  the  aorta  and  pulmonary  artery,  but  is  relatively  rich  in  muscular 
elements,  which,  as  well  as  the  elastic  tissue  are  known  to  undergo 
marked  increase  during  the  later  months  of  intra-uterine  life.     More 

1  Arch.  -Gen.  de  Med.,  1849,  xx,  415.  \These  de  Paris,  1862,  p.  67. 

^  Jena.  Zeitsch.  f.  Med.  u.  Naturwis.,  1867,  Bd.  iii. 
Westr.  Jahrb.f.  Padiatr.,  1871,  Bd.  i,  p.  1. 

5  Am.  Jour.  Med.  Sc,  1908,  pp.  136,  381. 

6  Univ.  of  Penn.  Med.  Bull.,  1910,  xxiii,  509.      ^  Gaz.  d.  hop.,  1910,  83,  p.  1419. 
8  Guy's  Hosp.  Gaz.,  May,  1901,  p.  197. 

^  Am.  Jour.  Med.  Sc,  1913,  cxlv,  543.  "  Trans.  Path.  Soc.  London,  ix,  48. 

^^Arch.  Middlesex  Hosp.  Clin.,  Series  XIII,  1913,  p.  39. 

^'' Johns  Hopkins  Hosp.  Bidl.,  1911,  xxii,  239. 

13  Edin.  Hosp.  Reports,  1900,  vi,  57. 

"  Proc.  Roy.  Soc.  Dis.  Ch.,  1909,  ii,  part  1,  p.  163. 

15  Viert.  f.  prak.  Heilk.,  1862,  ii,  92. 

1^  Guy's  Hosp.  Reports,  1873,  xviii,  22. 

"  Bull,  de  la  Soc.  Anat.  de  Paris,  1885,  x,  55. 

^^Zeit.f.  klin.  Med.,  1901,  xliii,  161. 

"  Wien.  klin.  Woch.,  1898,  xi,  p.  1195. 

20  Bull,  de  la  Soc.  Anat.,  February,  1907,  p.  179. 

^1  Ibid.,  1869,  xiv,  323.  22  QHn.  Soc.  Trans.,  1876,  ix,  152. 

^^Edin.  Med.  Journ.,  1900,  n.  s.,  viii,  1,  212,  436. 

^  Jour.  Am.  Med.  Assn.,  December  24,  1910. 


CONGENITAL  CARDIAC  DISEASE  411 

particularly  a  loose,  subintimal  layer  of  muscle  is  present  (Thoma^ ;  this 
evidently  corresponds  to  Jore's  musculo-elastic  layer  of  the  arterial  wall, 
which  is  here  developed  both  at  an  earlier  period  and  to  a  greater 
extent  than  in  the  aorta  and  pulmonary  artery.  It  is  especially  marked 
at  either  extremity  of  the  duct  where  it  can  be  seen  to  pass  into  and, 
indeed,  to  form,  the  musculo-elastic  layer  of  the  aorta  (Klotz).  When 
the  canal  is  emptied  of  contents,  as  happens  after  birth,  this  increased 
muscularity  enables  it  to  contract  firmly,  so  that  its  walls  remain  in 
juxtaposition  and  may  undergo  obliterative  endarteritis. 

2.  Alterations  in  the  Blood  pressure  at  Birth. — Previous  to  birth,  the 
pressure  is  highest  in  the  right  side  of  the  heart;  the  pulmonary  arteries 
are  small,  and  almost  all  the  blood  passes  through  the  ductus  into  the 
aorta.  At  birth  the  lungs  are  expanded,  their  capillaries  are  opened, 
and  there  is  an  immediate  lowering  of  pulmonary  blood  pressure.  Dr. 
Adami  suggests  that  during  the  first  few  days  of  life  the  aortic  tension, 
and  therefore  the  mean  blood  pressure  in  the  body  as  a  whole,  is  also 
lowered,  owing  to  the  reduced  amount  of  work  which  the  heart  is  called 
upon  to  perform,  after  the  cutting  off  of  the  placental  circulation,  and 
that  this  reduction  in  the  mean  blood  pressure  is  the  cause  of  the  collapse 
of  the  ductus,  and  the  main  factor  in  closure.  Kirstein  also  believes 
that  a  pressure  equilibrium  is  established  between  the  aortic  and  pul- 
monary circulations,  which  prevents  a  current  through  the  ductus  and 
thus  permits  of  its  obliteration,  while  Klotz  thinks  that  alterations  in 
the  pulmonary  pressure  at  birth,  and  the  relative  muscularity  of  the 
ductus  wall  are  together  sufficient  to  account  for  closure. 

3.  Nevertheless,  mechanical  conditions  preventing  the  flow  of  blood 
through  the  ductus  from  the  side  of  the  aorta  have  been  adduced  by  many 
workers,  and  may  reasonably  be  supposed  to  assist  in  the  process  of 
closure,  especially  when  the  pressure  in  the  aorta  comes  to  exceed  that 
in  the  pulmonary  circulation.  Schantz  supposed  a  stretching  of  the 
duct  by  the  movement  of  the  pericardium,  pulmonary  artery,  displaced 
thoracic  organs,  and  sternum,  in  the  initial  respiration,  and  Strassman 
described,  on  the  basis  of  a  large  number  of  injection  experiments,  a 
fold  in  the  aortic  wall  at  the  upper  border  of  the  mouth  of  the  duct, 
which  appears  about  the  seventh  month,  and  which  he  thinks  closes 
its  opening  in  a  valvular  manner  when  the  pressure  rises  in  the  aorta 
at  birth.  This  theory  of  a  valvular  aortic  fold  has  been  widely  accepted 
and  has  received  recent  confirmation  in  the  experimental  work  of  From- 
berg.2  Nevertheless,  its  constancy  in  infants,  and  its  valvular  action 
when  present,  has  been  gravely  disputed  by  such  careful  observers  as 
Klotz,  Kirstein  and  Stienon.^  The  last  word  on  this  subject  has  come 
from  Stienon,  who  has  found  that  Strassman's  fold  is  not  shown  on 
plaster  casts  of  the  ductus  and  adjacent  vessels  in  the  newly  born,  made 
under  low  pressure,  and  ascribes  its  appearance  postmortem  to  the  falling 
together  of  the  canal  after  its  evacuation.  From  the  study  of  a  large 
number  of  such  casts  of   the  aortic  isthmus  and  of    patent  ductus  at 

1  Virchows  Archiv,  1883,  vol.  xciii,  443. 

2  Baumgar.  Arbeit,  aus.  d.  Geb.  d.  Path.,  1914,  ix,  198. 

3  Archiv.  de  Biol.,  1912,  xxvii,  801. 


412  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

various  ages,  he  concludes  that  the  essential  mechanical  factor  in  closure 
is  the  dilatation  of  the  fetal  isthmus,  which  is  produced  by  raised  aortic 
tension  after  birth,  so  that  the  latter  has  the  secondary  effect  of  favoring 
closure  by  pressure  of  the  dilated  isthmus  on  the  aortic  end  of  the  duct. 
Dislocation  of  the  thoracic  organs  in  the  establishment  of  respiration 
probably  also  assists  in  diverting  the  circulation. 

From  the  above  considerations  the  general  conclusion  may  be  drawn 
that  continued  patency  will  occur  (1)  in  conditions  in  which  the  blood 
pressure,  either  in  the  aorta  or  pulmonary  artery,  is  maintained  at  a 
level  approximating  that  before  birth  (as  in  atelectasis  of  the  lungs), 
or  in  which,  for  any  other  cause,  a  high  positive  pressure  in  the  ductus 
is  maintained;  (2)  when  a  congenital  defect  in  the  structure  of  the 
ductus  wall  exists.  That  such  a  defect  is  not  uncommonly  the  cause  of 
patency  is  suggested  by  the  frequent  association  of  anomalies  elsewhere 
in  the  body  and  by  the  not  uncommon  occurrence  of  a  history  of  syphilis, 
or  of  anomalies  in  other  members  of  the  same  generation,  as  in  De  la 
Camp's  remarkable  series  of  six  brothers  and  sisters  all  with  characteristic 
physical  signs  of  patent  duct. 

That  raised  pulmonary  pressure  is  usually  at  fault  is  evidenced  by 
the  frequent  history  of  atelectasis  of  the  lungs,  diflBculty  in  suckling  or 
prolonged  delivery  in  the  mother,  seen  in  the  present  series.  In  support 
of  Stienon's  view,  that  dilatation  of  the  fetal  isthmus  is  an  important 
factor,  we  may  note  that  persistent  patency  is  associated  in  most  adult 
cases  with  a  certain  degree  of  coarctation  of  the  aorta,  and  that  the 
process  of  closure  after  birth  is,  like  dilatation  of  the  isthmus,  a  gradual 
one,  extending  over  the  first  weeks  of  life  and  often  not  completed  until 
the  third  month. 

Pathology. — Three  principal  types  of  patency  may  be  distinguished: 
(1)  The  duct  may  be  greatly  shortened  upon  itself  so  that  its  ends  are 
approximated  to  each  other,  and  it  disappears  as  a  canal,  remaining 
as  a  simple  aperture  between  the  two  great  trunks.  (2)  More  fre- 
quently the  ductus  persists  as  a  short  canal  from  0.4  to  2  cm.,  long 
(Vierordt),  with  a  lumen  varying  in  size  from  one  just  admitting  a  bristle 
to  one  allowing  the  passage  of  a  "goose-quill,"  "pencil,"  or  even,  as  in 
Luys'  case,  the  "finger."  A  patent  ductus  of  long  standing  is  usually 
shorter  and  broader  than  that  of  infancy  or  later  fetal  life.  In  form  this 
canal  may  be  (a)  cylindrical,  as  is  usual  in  infants,  and  as  was  seen  in 
the  cases  by  Fagge,  Almagro,  Gerhardt,  and  White^  in  adults;  or  (b) 
funnel-shaped  (i.  e.,  conical,  as  in  a  funnel  without  a  stem),  with  its 
larger  end  toward  the  aorta,  as  in  a  case  by  Murray,  in  which  in  a 
woman  aged  thirty-six  years,  it  formed  a  truncated  cone  three-eighths 
of  an  inch  long,  just  admitting  a  quill,  and  lying  with  its  base  to  the 
aorta.  Finally  (following  Gerhardt's  classification  into  four  types,  of 
which  the  above  forms  1,  2a  and  h  constitute  the  first  three),  the  patent 
duct  may  exist  (3)  as  a  canal  which  has  undergone  aneurismal  dilatation. 

In  a  patent  ductus  with  otherwise  normal  conditions,  the  blood  stream 
will  be  directed  chiefly  from  the  aorta,  where  the  blood  pressure  is 

1  Trans.  Path.  Soc,  London,  1885,  xxxvi,  182. 


PLATE  VI 


B~ 


.  •'  '\ 


D-  - 

F 


*^ 


:;i^::>- 


-c 


H 


y' 


P.A. 


fe. 


Microscopic  appearances  of  wall  of  patent  ductus  and  adjacent  aorta  and  pulmonary  artery 
in  a  case  of  patent  ductus  arteriosus  with  acute  infective  pulmonary  endarteritis,  showing 
site  of  initial  lesion  at  pulmonary  end  of  ductus.  Drs.  Hamilton  and  Abbott.  (Colored  draw- 
ing by  Dr.  J.  H.  Atkinson.)  (Haem.  and  eos.  and  elastic  tissue  stains.  Low  magnification.) 
A  rectangular  block  has  been  cut  to  include  the  whole  wall  of  the  ductus  (D,  D')  and  a  portion 
of  the  piilmonary  artery  (P.A.)  and  aorta  {A)  adjacent.  A,  wall  of  aorta  which  is  quite 
healthy.  P.A.,  wall  of  pulmonary  artery.  D,  D',  wall  of  ductus  arteriosus  in  which  the  elastic 
tissue  is  almost  destroyed,  and  which  is  surmounted  by  a  thrombotic  mass  {B) .  C,  pulmonary 
end  of  ductus,  showing  destruction  of  elastica,  and  organization  of  inflammatory  products 
(z.  e.,  seat  of  initial  lesion).  E,  aortic  end  of  ductus  showing  zone  of  recent  inflammatory 
exudate  and  invasion  of  tissue  between  aorta  and  piolmonary  artery  by  acute  inflammation. 
F,  necrosed  area  below  ductus  wall.  G,  recent  acute  inflammatory  process  extending  from 
aortic  end  of  ductus  into  cellular  tissue  between  aorta  and  pulmonary  artery.  H,  thrombotic 
mass  overlying  wall  of  pulmonary  artery  and  becoming  incorporated  with  it  in  neighborhood 
of  piilmonary  end  of  ductus. 


CONGENITAL  CARDIAC  DISEASE  413 

higher,  into  the  pulmonary  artery.  This  is  evidenced  by  the  funnel- 
shaped  form  with  its  base  toward  the  aorta,  which  the  canal  usually 
assumes  in  adults,  and  by  the  presence  of  mycotic  vegetations  on  the 
adjacent  wall  of  the  pulmonary  artery  in  all  the  cases  of  acute  infective 
endarteritis  in  the  neighborhood  of  a  patent  duct.  Wagener's  3  cases, 
in  which  the  membrane  at  the  pulmonary  end  bulged  into  the  artery, 
also  indicate  this  direction  of  the  stream.  Dilatation  of  the  pulmonary 
artery,  and  hypertrophy  and  dilatation  of  the  right  ventricle,  are  usual 
results  of  patency  of  long  standing.  Rauchfuss  thought  them  character- 
istic of  all  cases,  but  exceptions  occur.  The  left  ventricle  may  share  in 
the  hypertrophy  and  the  aorta  be  moderately  dilated.  In  Fagge's 
case,  a  woman  aged  forty-two  years,  the  right  ventricle  was  greatly 
hypertrophied,  being  equal  to  the  left  in  thickness;  the  right  auricle 
was  dilated,  and  the  main  pulmonary  branches,  especially  the  right, 
were  much  dilated.  The  left  ventricle  is  occasionally  hypertrophied  in 
excess  of  the  right.  In  rare  instances,  as  in  Walsham's  and  Drasche's 
cases,  aged  respectively  forty-seven  and  twenty-nine  years,  the  heart 
may  not  be  hypertrophied  at  all. 

Arteriosclerotic  patches  are  not  uncommon  in  the  neighborhood  of 
the  patent  duct  in  the  aorta,  and  extensive  atheroma  may  occur  also 
in  the  pulmonary  artery.  In  Hebb's^  case  the  atheroma  and  dilatation 
of  this  trunk  seem  to  be  explained  rather  by  the  obliteration  of  its  left 
branch  through  the  pressure  of  the  thrombosed  duct. 

Durno  and  Brown,^  report  a  case  in  a  man  of  thirty-three,  of  widely 
patent  ductus  arteriosus,  with  extensive  atheroma  both  of  the  walls 
of  the  ductus,  and  of  the  greatly  dilated  pulmonary  artery.  A  small 
saccular  aneurism  of  the  pulmonary  close  to  the  ductus  had  ruptured, 
forming  a  dissecting  aneurism  which  in  turn  burst  into  the  pericardium. 

Acute  Infective  Pulmonary  Endarteritis. — Vegetations  of  a  malig- 
nant character  are  not  uncommon  within  a  patent  duct,  about  its  aortic 
orifice,  and  on  the  adjacent  wall  of  the  pulmonary  artery.  There  are 
13  such  cases  in  our  series;  in  all,  the  pulmonary  artery  adjacent  to 
the  ductus  was  extensively  diseased,  and  in  all  but  one  (Hamilton  and 
Abbott^),  the  heart  valves  were  also  involved  in  a  malignant  endocar- 
ditis. This  last  case  was  of  especial  interest  because  of  the  strict  local- 
ization of  the  infective  process  to  the  ductus  and  the  pulmonary  artery 
adjacent,  which  showed  clearly  that  the  acute  inflammatory  process 
had  originated  in  the  immediate  neighborhood  of  the  defect,  a  point 
confirmed  by  microscopic  examination,  in  which  the  organization  of 
the  inflammatory  products  proceeding  at  the  pulmonary  end  of  the 
ductus  was  clearly  seen  (see  Plate  VI),  thus  demonstrating  this  to  have 
been  in  all  probability,  the  earliest  initial  seat  of  a  process,  which  had 
elsewhere  and  later  assumed  a  fulminating,  highly  destructive  character. 

The  'patient  w^as  a  girl  of  nineteen  years,  who  presented  a  clinical 
picture  of  septicemia  for  some  weeks  before  death,  and  the  characteristic 
physical  signs  of  patent  ductus,  without  valvular  involvement,  or  cyanosis. 
Postmortem  a  huge  thrombotic  mass  of  vegetations  lay  in  the  lumen  of 

1  Trans.  Path.  Soc,  London,  vol.  xliv,  45.  -  Lancet,  1908,  i,  1692. 

^  Trans.  Assoc.  Am.  Phys.,  1914,  vol.  xxix  (gives  full  bibliography). 


414 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


the  dilated  pulmonary  artery  blocking  the  orifice  of  a  large  patent  duc- 
tus, and  extending  into  the  left  pulmonary  artery  (see  Fig.  38).  The 
aorta  was  stenosed  at  the  isthmus  but  was  otherwise  healthy  and  the 
endocardium  of  the  heart  was  free  from  every  trace  of  disease.  Embolic 
abscesses  in  the  lungs,  the  vegetations  in  the  pulmonary  artery,  and  the 
blood  culture  during  life  contained  swarms  of  pneumococci.  Both  the 
patent  ductus  and  pulmonary  endarteritis  were  diagnosed  during  life. 


Fig.  38 


XOnXII'C   FROK 
B  E  a  I  3  D 


coKsa 


/^i  % 


Diagrammatic  drawing  showing  acute  vegetative  endarteritis  of  pulmonary  artery  in  the  neighborhood 
of  the  patent  ductus  arteriosus,  and  consequent  infarcts  of  the  lung.  A  probe  is  seen  passed  through 
the  patent  ductus.     (W.  F.  Hamilton  and  M.  E.  Abbott.) 

The  wall  of  the  aorta  opposite  the  ductus  was  the  seat  of  the  mycotic 
vegetations  in  some  cases,  indicating  that  the  infection  had  proceeded 
with  the  current  through  the  ductus  and  had  impinged  here. 

Paradoxical  Embolism. — In  Schmorl's^  case,  an  embolus  passed  from 
a  primary  thrombus  in  the  left  auricle,  through  a  patent  ductus  arteriosus 
to  the  pulmonary  artery.  In  the  cases  of  acute  infective  pulmonary 
endarteritis  enumerated  above,  septic  infarcts  in  both  systemic  and 
pulmonary  circulations,  evidently  from  emboli  passing  through  the 
patent  ductus,  were  extremely  common,  occurring  even  in  those  cases 
in  which  only  the  tricuspid  valve  and  pulmonary  artery  were  diseased. 
Hochhaus  based  a  correct  diagnosis  upon  this  feature. 

Symptoms  and  Signs. — Clinical  evidence  of  patency  of  the  ductus 
is  to  be  sought  rather  in  physical  signs  than  in  symptoms,  for  the  latter 
are  often  obscure.     Nevertheless,  their  very  negative  character  when 


Verhandl.  d.  deutsch.  path.  Gesellsch.,  1909,  xiii,  217. 


CONGENITAL  CARDIAC  DISEASE  415 

taken  in  combination  with  the  distinctly  characteristic  physical  signs, 
presents,  in  the  majority  of  cases,  adequate  grounds  for  a  correct  diag- 
nosis, and  this  can  almost  always  be  made.  Careful  contributions  are 
now  numerous,  and  a  symptom  complex  has  been  built  up  which  makes 
this  chapter  in  congenital  defects  almost  as  legible  to  the  clinician  as 
that  of  any  form  of  acquired  cardiac  disease.  On  account  of  the  secondary 
anatomical  changes  that  are  usually  induced  in  a  patent  ductus  of  long 
standing,  such  as  shortening  and  widening  of  the  duct  and  dilatation  of 
the  pulmonary  artery,  the  picture  in  infants  and  early  childhood,  is 
somewhat  diflferent,  and  much  less  distinctive  than  that  in  later  life. 
This  statement  applies  especially  to  the  physical  signs. 

The  typical  appearance  is  one  of  anemia,  sometimes  profound,  which 
has  been  described  as  wax-like.  Cyanosis  is  usually  absent;  when 
present  it  is  generally  slight  and  transient,  appearing  only  on  exertion, 
and  usually  develops  late,  sometimes  as  a  terminal  event.  Of  the  57 
cases  in  which  this  point  is  mentioned,  cyanosis  was  entirely  absent  in 
27,  of  which  19  were  in  adults  and  8  were  in  children  under  two  years. 
Cyanosis  was  noted  as  slight  in  13  cases,  in  one  of  which,  Bittorf's  (aged 
eleven  years),  it  was  constant,  in  the  others  transient,  appearing  only 
on  crying  in  Simmons'  (aged  sixteen  weeks),  and  during  anginal  attacks 
in  Hale  White's  case  (aged  fifty-three  years).  It  was  moderate  in  4 
cases  only.  In  that  by  Carmichael,  dying  at  three,  it  came  on  soon  after 
birth,  becoming  extreme,  with  clubbing  and  a  polycythemia  of  8,100,000. 
Coarctation  of  the  aorta  was  associated  with  the  patent  duct,  and  mitral 
stenosis  with  great  dilatation  of  the  left  auricle  was  present  as  well, 
suggesting  a  rise  of  pressure  in  the  pulmonary  artery  and  a  possible 
reversal  of  flow,  venous  blood  from  this  vessel  entering  the  aorta  through 
the  canal. 

Dyspnoeic  attacks  usually  accompanied  by  transient,  but  marked 
cyanosis,  are  relatively  common  in  infants  and  are  so  characteristic  of 
these  cases  that  the  name  La  Cyanose  Congenitale  Paroxystique  has 
been  proposed.  Loss  of  consciousness  may  occur  during  the  attack  and 
the  heart  may  stop  beating,  or  death  may  supervene.  Three  typical 
cases  were  reported  by  Hall  in  infants,  all  of  whom  died  during  the 
attack,  and  others  by  Sanders,  Carmichael,  Luys,  and  Bommer.^  In 
the  latter  (aged  sixteen  weeks)  the  cyanosis  was  transient,  coming  on 
only  during  the  attacks,  which  came  on  especially  during  feeding,  and 
recurred  at  last  so  frequently  that  the  child  failed  for  lack  of  nourishment; 
during  the  attacks  the  breathing  stopped  suddenly  and  deep  cyanosis 
developed,  lasting  two  to  four  minutes;  it  passed  off  entirely  as  the 
breath  returned,  in  the  interval  the  color  being  normal.  This  is  very 
suggestive  of  an  admixture  of  venous  with  arterial  blood  as  the  cause 
of  temporary  cyanosis,  the  pressure  becoming  higher  in  the  pulmonary 
artery  and  lower  in  the  aorta  during  the  act  of  suckling. 

In  older  subjects  cardiac  seizures  of  various  sorts  may  replace  these 
suffocative  dyspnoeic  attacks.  Paroxysms  of  extreme  tachycardia 
(pulse  200),  with  dyspnoea  and  bloody  expectoration,  lasting  for  some 

1  Freiburg  Thesis,  1900. 


416  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

hours,  and  recurring  every  few  months,  are  described  in  a  man  aged 
thirty-six  years,  with  dyspnoea  and  palpitation  on  exertion  for  years, 
but  no  cyanosis  (Bommer).  Hale  White  reports  repeated  angina-like 
attacks,  in  one  of  which  death  occurred,  in  a  man  aged  fifty-three  years, 
with  a  patent  duct  the  size  of  the  anterior  tibial  artery,  but  no  hyper- 
trophy of  the  heart  or  disease  of  this  or  of  the  aorta. 

Epistaxis,  hematemesis,  and  hemorrhages  from  other  mucous  surfaces 
are  not  uncommon  (Almagro,  Carmichael,  Darier,  Duroziez).  Unless 
death  occurs  from  some  intercurrent  condition,  as  malignant  endocar- 
ditis or  endarteritis,  the  patients  usually  die  with  failing  compensation, 
and  dyspnoea  is  a  remarkably  constant  feature.  Sudden  death  occurred 
in  eleven  cases  in  our  series;  in  six  during  dyspnoeic  attacks,  in  three, 
those  by  Reid,  Crouzet  and  Chessman,  without  apparent  cause,  in  the 
case  by  Mead  from  rupture  of  the  heart  and  in  that  by  Durno  Brown 
from  rupture  of  the  pulmonary  aneurism  above  described. 

Physical  Sigm  are  almost  invariably  present,  and  are  usually  charac- 
teristic in  older  patients.  In  infants  they  are  practically  indistinguishable 
from  those  produced  by  auricular  and  ventricular  septal  defects.  This 
is  because  the  patent  duct  is  at  first  a  straight  canal,  which  does  not 
allow  of  the  passage  of  a  large  volume  of  fluid  and  because  in  the  absence 
of  dilatation  of  the  pulmonary  artery  there  is  less  sound  produced  by 
the  impinging  of  currents  in  this  situation.  Among  our  64  cases  physical 
signs  were  absent  in  only  8  cases.  Absence  of  physical  signs  in  the  case 
of  Walsham,  quoted  by  Vierordt  in  this  connection,  must  be  pronounced 
doubtful,  for  the  specimen  came  from  the  dissecting-room  with  an  indefi- 
nite note  that  cyanosis  and  pericardial  murmurs  existed.  A  negative 
finding  in  the  cases  by  Luys  and  Duroziez  was  also  disputed  by 
Almagro. 

The  distinctive  physical  signs  (which  develop  as  life  proceeds),  as 
well  as  the  absence  or  late  appearance  of  cyanosis,  depend,  as  Gerhardt 
pointed  out,  on  the  fact  that  a  patent  duct  of  long  standing  usually  has 
a  short,  wide  lumen  through  which  during  systole  blood  flows  freely 
from  the  aorta  into  the  pulmonary  artery,  which  dilates  accordingly  and 
becomes,  with  the  ductus  itself,  the  chief  seat  of  whatever  vibration  or 
murmur  the  abnormal  current  may  produce;  the  right  ventricle  behind 
it  usually  undergoes  hypertrophy  and  dilatation  as  well.  Gerhardt 
described  as  characteristic  a  visible  systolic  pulsation  in  the  second 
left  interspace  (indicating  the  forcible  closure  of  the  pulmonary  valves), 
an  increased  area  of  cardiac  dulness,  especially  to  the  right,  and  a  narrow 
zone  of  dulness  3  to  4  cm.  wide  (corresponding,  he  believed,  to  the  dilated 
pulmonary  artery)  lying  at  the  base  of  the  heart,  along  the  left  sternal 
border  from  the  third  to  the  second  or  first  rib,  and  extending  a  little 
way  over  the  first  piece  of  the  sternum.  This  "ribbon-shaped"  dulness 
has  been  noted  by  many  other  observers,  and  has  recently  been  strikingly 
confirmed  in  a  number  of  cases  in  which  Gerhardt's  dull  area,  with 
characteristic  murmur  or  thrill  localized  over  it,  has  been  found  by  the 
a--ray  to  correspond  with  a  pulsating  shadow  lying  above  the  base  of 
the  heart,  which  was  evidently  from  its  size  and  position  the  dilated 
pulmonary  artery.    In  Bittorf's  case  this  shadow  was  seen,  when  looked 


CONGENITAL  CARDIAC  DISEASE  417 

at  from  the  side,  to  be  the  size  of  a  walnut  and  to  pulsate  a  little  later 
than  the  heart  and  synchronously  with  the  aorta.  In  Arnheim's  case 
the  a'-rays  showed,  besides  enormous  hypertrophy  of  both  sides  of  the 
heart,  which  occupied  nearly  the  whole  left  thorax,  the  greatly  enlarged 
shadow  of  the  pulmonary  artery  placed  above  the  cardiac  shadow  "like 
a  cap,"  and  numerous  tortuous  dilated  vessels,  indicating  an  extensive 
collateral  circulation  and  a  probable  coexisting  coarctation  of  the  aorta. 
In  the  cases  reported  by  Schrotter,  Mead,  and  Hamilton  and  Abbott, 
the  .T-ray  cap,  and  Gerhardt's  dulness,  were  found  at  autopsy  to  corre- 
spond vnth  the  dilated  pulmonary  artery. 

When  cardiac  hypertrophy  is  marked,  precordial  bulging,  diffuse 
pulsation,  and  other  evidences  will  be  present.  An  increased  area  of 
cardiac  dulness,  especiallv  to  the  right,  while  usual,  is  not  invariable,  for 
the  left  ventricle  may  b/hypertrophied  in  excess  of  the  right  (Murray's 
case),  or  in  rare  instances  there  may  be  no  cardiac  hypertrophy  at  all 
(Drasche's  case). 

A  thrill,  usually  systolic,  but  sometimes  continuous  through  the  cardiac 
cycle,  is  fairly  frequent,  and  was  present  in  17  of  the  64  cases.  It  may  be 
diffuse  over  the  precordium,  but  is  usually  localized  to  the  neighborhood 
of  the  second  left  interspace,  in  the  region  described  above  as  Gerhardt's 
dull  area,  or  at  least  is  of  maximum  intensity  here.  Its  transmission 
obliquely  upward  below  the  clavicle  (along  the  course  of  the  pulmonary 
artery)  is  said  to  be  pathognomonic. 

The  auscultatory  phenomena  are  the  most  important  and  constant.  In 
infants  a  harsh  systolic  murmur  with  more  or  less  of  the  above  localiza- 
tion is  the  rule,  but  in  adults  a  loud  murmur  is  nearly  always  produced, 
which  is  characterized  by  almost  all  observers  as  peculiar,  and  is  variously 
described  as  harsh,  musical,  scraping,  scratching,  humming,  churning, 
rushing,  rolling,  and  only  rarely  as  blowing.  Miiller  compares  it  in  his 
case  to  "rolling  thunder,"  and  says  that  two  different  listeners  likened 
it  independently  to  the  noise  made  by  a  train  in  passing  through  a  tunnel, 
and  Thayer  described  it  in  Mead's  case  as  a  "machinery  murmur." 
In  rhythm  several  different  t>^es  may  be  made  out:  (1)  The  murmur 
is  frequently  systolic  (as  in  the  cases  by  Murray,  Hale  White,  Simmons, 
and  Bittorf).  (2)  It  may  begin  with  systole,  but  continue  into  and 
through  diastole,  either  as  a  continuous  hum  (Chessman's  case), 
or  with  a  systolic  rise  (Bommer),  or  with  a  rhythmic  systolic,  and 
diastolic  accentuation.  Gibson^  describes  as  pathognomonic  a  con- 
tinuous, rushing  murmur  which  "begins  distinctly  after  the  first 
sound,  accompanies  the  latter  part  of  that  sound,  occupies  the  first 
pause,  accompanies  the  second  sound  (which  may  be  accentuated  in 
the  pulmonary  area,  or  doubled),  and  finally  dies  away  during  the  long 
pause."  (3)  Sometimes,  as  in  Drasche's  case,  two  independent  murmurs 
are  heard  at  the  pulmonary  area,  the  loud,  peculiar,  systolic  one,  and  a 
low,  short,  diastolic,  indicating  a  slight  regurgitation  into  the  aorta 
during  the  pause.  (4)  More  rarely  the  murmur  is  diastolic  in  rhythm, 
as  in  Fagge's  case,  in  which  a  diastolic  murmur,  musical  and  of  a  wavy 

1  Medical  Press  and  Circular,  May  30,  1906. 
VOL.  IV — 27 


418  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

character,  was  localized  to  the  pulmonary  cartilage.  The  point  of  maxi- 
mum intensity  is  usually  in  the  second  or  third  left  interspace,  and  it 
is  often  heard  very  loudly  in  the  first  left  interspace  below  the  clavicle 
and  over  the  first  part  of  the  sternum  and  in  the  back  to  the  left  of  the 
third  and  fourth  dorsal  vertebrae,  and  in  the  left  suprascapular  region. 
In  Franck's  case  and  in  one  by  Gillet,  the  murmur  was  only  heard  pos- 
teriorly in  this  situation,  and  not  in  front  at  all.  It  is  transmitted  over 
the  left  ventricle,  and  its  systolic  element  often  is  audible  over  the  carotids, 
sometimes  more  distinctly  over  the  left  than  over  the  right  (Gerhardt). 
It  may  diminish  abruptly  below  the  third  left  costal  cartilage.  In  this 
series  of  64  cases,  among  the  18  cases  in  infants,  in  8  a  systolic,  and  in  1 
a  continuous  murmur  was  present.  Among  the  46  adults,  in  19  cases 
the  murmur  was  systolic,  in  9  "double"  and  in  14  it  was  the  continuous 
''harsh,"  "rumbling,"  "rolling,"  "churning,"  "humming"  murmur 
usually  with  systolic  accentuation,  described  by  the  earliest  students 
of  this  subject  as  characteristic,  and  which  Gibson  rightly  described  as 
pathognomonic. 

Franck  mentions,  as  of  diagnostic  value,  an  inspiratory  accentuation 
and  an  expiratory  diminution  both  of  the  characteristic  murmur  and  of 
the  radial  pulse  (pulsus  paradoxus),  which  they  explain  by  saying  that 
during  respiration  the  pressure  in  the  thorax  is  lowered,  so  that  more 
blood  can  enter  the  pulmonary  artery  then  than  during  expiration,  and 
this  will  lead  to  a  smaller  pulse  wave  from  the  aorta,  to  a  larger  current 
through  the  canal,  and  a  correspondingly  louder  murmur. 

The  second  pulmonary  sound  is  frequently  much  accentuated,  and 
this  is  very  important  as  distinguishing  patency  of  the  duct  from  pul- 
monary stenosis  with  somewhat  similar  localization  of  murmur  or  thrill. 
On  the  other  hand,  in  some  cases  it  may  be  weak  or  even  inaudible. 

In  the  cases  by  Schrotter  and  Mead  paralysis  of  the  left  recurrent 
laryngeal  nerve  was  present,  due  to  pressure  upon  the  nerve  by  the  en- 
larged patent  duct.  Schrotter  based  a  correct  diagnosis  on  this  feature. 
The  nerve  was  degenerated  on  microscopic  examination. 

The  physical  signs  are  very  often  obscured  by  those  of  other  lesions, 
as  malignant  endocarditis  or  arteritis,  chronic  valvular  disease,  or  other 
cardiac  anomalies  so  commonly  associated.  The  peculiar  character  of 
'the  murmur,  its  more  or  less  prolonged  rhythm,  its  localization,  and 
that  of  the  thrill  when  present,  high  up  toward  the  left  infraclavicular 
region,  with  the  results  of  .r-ray  examination,  remain,  even  in  these 
complicated  cases,  of  the  first  diagnostic  value.  Patent  duct  must  be 
diagnosed  also  from  perforation  of  the  aorta  and  pulmonary  artery  just 
above  the  semilunar  valves,  whether  oif  inflammatory  or  congenital 
origin.     Brocq^  gives  a  long  series  of  cases  of  both  types. 

So-called  aneurisms  of  the  ductus  Botalli  give  rise  to  no  physical 
signs,  being  of  small  size  and  usually  occluded  by  thrombus.  They  are 
generally  said  to  be  of  little  clinical  significance,  but  death  from  rupture 
of  their  walls  has  been  reported  and  embolism  from  the  thrombus  within 
may  lead  to  a  fatal  result. 

1  Rev.  de  Med.,  1886,  vi,  786. 


CONGENITAL  CARDIAC  DISEASE  419 

So-called  Aneurisms  of  the  Ductus  Botalli. — This  term  is  used  in  the 
literature  with  a  rather  irregular  application  to  denote  a  dilatation  in 
whole  or  in  part  of  a  persistently  patent  duct.  That  the  cases  are  not 
aneurisms  in  the  strict  sense  is  inferred  by  most  writers.  Rokitansky 
uses  the  qualifying  word  "so-called."  Gruner  says  that  arterial  dilata- 
tion would  often  be  a  better  word,  as  there  is  usually  no  change  in  the 
vessel  wall,  and  he  draws  attention  to  the  fact  that  in  the  usual  bean- 
shaped  form  the  constriction  at  either  end  makes  the  ductus  appear 
larger  to  the  eye  than  it  really  is.  Klotz  has  suggested  that,  as  in  his 
injection  experiments  the  duct  is  seen  to  be  much  larger  at  birth  when 
distended  with  fluid  than  when  contracted  at  the  autopsy,  many  of 
these  small,  so-called  aneurisms,  measuring  less  than  1  cm.  in  their 
greatest  diameter,  are  really  not  even  a  dilatation,  but  are  a  simple 
distension  of  a  patent  duct  to  its  full  capacity  by  the  coagulum  within. 
Again,  a  further  confusion  exists  in  that  the  term  is  applied  more  widely 
by  some  writers  than  by  others.  Nevertheless,  the  cases  recorded  form 
a  fairly  well-defined  group,  which,  from  their  rarity  and  from  the  fact 
that  the  duct  is  usually  occluded  by  thrombus,  are  chiefly  of  pathological 
interest,  although  their  occasional  rupture,  and  also  the  risk  of  embolism 
from  the  thrombus  within,  increase  their  clinical  significance.  The  first 
cases  reported  were  by  Billard,  Thore,  and  other  French  writers;  Roki- 
tansky  followed  with  his  monograph  in  1852,  and  Virchow  in  1856; 
full  studies  of  the  literature  with  original  cases  are  to  be  found  in  the 
theses  of  Westhoff,^  and  Gruner.^ 

In  what  may  be  taken  as  the  classical  form  (which  is  that  described  by 
Rokitansky)  the  ductus  forms  a  spherical  or  ovoid  tumor  larger  at  the 
middle  than  at  either  end,  but  smallest  toward  the  pulmonary  artery, 
with  which,  as  well  as  with  the  aorta,  it  communicates,  filled  with  old 
or  recent  thrombus,-and  varying  in  size  from  a  "cherry  stone"  (Billard) 
to  a  "hazel  nut"  (Thore),  or  even  a  walnut  (Hebb,  Binzer).  In  Hebb's 
case,^  in  a  man  aged  forty  years,  an  aneurism  the  size  of  a  small  walnut, 
filled  with  old  clot,  lay  in  the  position  of  the  duct,  communicating  with 
the  aorta  by  an  orifice  one-eighth  of  an  inch  in  diameter,  and  abutting 
against  the  obliterated  left  pulmonary  artery  and  left  bronchus.  All  the 
cases  recorded  are  in  infants  excepting  that  by  Hebb. 

In  Thoma's*  patient,  aged  twenty-six  years,  the  aorta,  from  the 
isthmus  downward  for  about  4  cm.,  was  dilated  in  the  form  of  a  spindle, 
was  lined  by  atheromatous  plaques,  and  on  its  right  wall  opposite  the  left 
subclavian  artery  was  a  saccular  aneurism,  in  the  floor  of  which  lay  a 
small  hole  representing  the  lumen  of  the  greatly  shortened  ductus  lead- 
ing into  the  pulmonary  artery,  which  was  here  firmly  adherent  to  the 
aorta.  Microscopic  examination  showed  this  aneurism  not  derived  from 
an  expansion  of  the  aortic  end  of  the  ductus,  but  to  be  a  bulging  of  the 
aortfc  wall,  which  the  writer  thought  was  pulled  to  the  right  by  the 
action  of  the  contracted  ductus.  Rokitansky's  5  cases  of  funnel-shaped 
patency  were  explained  by  Thoma  in  the  same  way,  and  a  special  form 
of  "traction  aneurism  of  the  infantile  aorta"  was  thus  established  by 

1  Gottingen  Diss.,  1873  (quoted  by  Gruner  and  Voss).  -  Freiburg  Diss.,  1904. 

^  Trans.  Path.  Soc,  London,  1893,  xliv,  45. 
4  Virchows  Archiv,  1890,  Bd.  122,  p.  535. 


420  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

him.  In  Wagener's  3  cases,  aged  respectively  thirty-eight,  forty-two, 
and  twenty-three  years,  the  duct  formed  a  distinct  canal  with  a  small 
lumen  open  on  the  side  of  the  aorta,  where  the  orifice  lay  in  the  floor  of 
a  hollow  in  the  wall  of  this  vessel,  and  was  sheltered  by  a  distinct  fold 
of  aortic  intima  projecting  downward  from  above  while  the  pulmonary 
end  was  closed  in  by  a  thin  membrane,  which  bulged  into  the  pulmonary 
artery.  Mycotic  aneurism  of  the  patent  duct  of  the  dissecting  form 
has  been  described  by  Buhl. 

Absence  of  the  Ductus. — Absence  of  the  ductus  may  occur,  and  is 
usually  associated  with  hypoplasia  and  shortening,  sometimes  with 
atresia,  of  the  pulmonary  artery.  It  is  explained  as  due  to  a  primary 
failure  of  development  of  the  sixth  left  branchial  arch  (which  persists 
as  the  ductus),  the  stenosis  of  the  pulmonary  being  secondary.  In  these 
cases  a  septal  defect  is  present,  through  which  the  aerated  blood  passes 
from  the  right  heart  to  the  aorta. 

Anomalous  Course. — Multiple  origin  is  reported  by  Peacock  in  a  case 
of  pulmonary  stenosis,  two  small  trunks  arising  at  the  site  of  the  normal 
ductus  and  passing,  the  smaller  into  the  left,  the  larger  (which  was  cut 
short)  apparently  into  the  right  pulmonary  artery.  In  several  cases  the 
canal  has  opened  into  the  left  subclavian.  In  one  case,  of  right  aortic 
arch,  the  duct  entered  the  descending  aorta  below  the  right  subclavian 
and  itself  gave  off  the  left  subclavian  artery. 

COARCTATION    OF    THE    AORTA. 

This  term  applies  to  a  well-recognized  group  of  cases  in  which  there  is 
a  narrowing  or  stenosis,  amounting  sometimes  to  a  complete  obliteration, 
of  the  descending  arch  at,  or  immediately  below,  the  so-called  isthmus 
of  the  aorta,  which  is  that  part  of  this  vessel  lying  between  the  left  sub- 
clavian artery  and  the  insertion  of  the  ductus  arteriosus.  During  the 
period  of  fetal  circulation  this  segment  is  comparatively  little  used,  and 
at  birth  is  usually  observed  to  be  of  slightly  smaller  lumen  than  the 
adjacent  portions  of  the  aorta,  the  difference  soon  disappearing  under 
normal  conditions.  Theremin  states,  as  a  result  of  his  measurements  of 
the  normal  infant  heart,  that  in  SO  per  cent,  a  slight  diminution  in 
diameter  exists  in  the  isthmus  during  the  first  three  months  of  postnatal 
life,  after  which  a  calibre  uniform  with  the  remainder  of  the  arch  is 
attained;  and  that  in  some  6  per  cent,  a  slight  difference  remains 
throughout  life  which  he  does  not  consider  abnormal  unless  it  amounts 
to  more  than  2  mm.  Bonnett  classed  as  anomalous  those  cases  in  which 
the  difference  was  about  3  mm. 

Two  distinct  groups  of  cases  are  understood  under  the  term,  (a)  A 
diffuse  narrowing  of  the  aorta  at  the  isthmus  (Bonnet's  infantile  type). 
In  some  of  these  cases  in  which  the  stenosis  is  marked,  the  circulation  in 
the  lower  part  of  the  body  is  maintained  by  a  large  patent  ductus  arterio- 
sus through  A^hich  the  descending  aorta  aypears  to  he  a  direct  continuation 
of  the  jjvhnonary  artery.  Such  cases,  being  essentially  the  same  in  origin 
as  coarctation,  may  be  included  with  it.  (h)  A  more  or  less  abrupt  con- 
striction of  the  aorta  at  or  near  the  insertion  of  the  ductus  arteriosus 
(Bonnet's  adult  type).     Here,  where  coarctation  is  marked  and  has 


CONGENITAL  CARDIAC  DISEASE  421 

lasted  some  time,  the  establishment  of  an  extensive  collateral  circulation 
frequently  completes  the  picture  and  lends  distinctive  features  to  what 
is  otherwise  an  obscure  lesion. 

Relative  Frequency. — The  figures  in  the  literature  are  somewhat  mis- 
leading, for  curiously  little  account  is  taken  of  its  occurrence  by  many 
workers,  and  therefore  the  lesser  degrees  of  coarctation  are  probably 
often  overlooked  in  the  postmortem  room,  and  cases  with  well-marked 
vascular  changes  may  escape  diagnosis  at  the  bedside.  On  the  other 
hand,  this  subject  has  been  so  carefully  worked  over  and  brought  up  to 
date  by  successive  writers,  that  its  statistics  are  clearer  and  more  acces- 
sible than  is  the  case  perhaps  in  any  other  chapter  of  congenital  cardiac 
disease.  Very  probably,  therefore,  the  212  cases  enumerated  here  are 
not  far  from  being  the  full  number  recorded,  whereas  the  total  number 
of  pulmonary  stenosis  or  of  septal  defect  (which  anomalies  have  not 
been  subjected,  at  least  of  recent  years,  to  such  careful  repeated  statis- 
tical analysis)  must  be  much  higher  than  that  given  by  any  author. 
For  this  reason  Vierordt's  statement  that  coarctation  ranks  next  in 
frequency  to  pulmonary  stenosis  is  probably  placing  the  incidence  too 
high.  A  truer  estimate  may  perhaps  be  gathered  from  the  fact  that 
among  205  cardiac  anomalies  recorded  in  the  Transactions,  there  are 
22  of  stenosis  or  obliteration  of  the  aorta  at  the  isthmus  and  2  of  entire 
absence  of  the  aortic  trunk  between  the  left  subclavian  and  the  ductus, 
against  91  of  pulmonary  stenosis  and  165  defects  of  the  interventricular 
septum. 

The  first  case  was  reported  by  Paris  in  1789.  Craigie  collected  10 
from  the  literature  in  1841,  von  Leeuwen  18  in  1850,  Rokitansky  26 
in  1852,  and  Peacock  46  cases  in  1866.  Barie,'^  in  1885,  gave  a  review  of 
89  cases,  in  which  he  published  the  series  of  the  above  authors,  with 
others  from  the  literature.  The  fact  that  6  of  these  are  without  autopsy 
findings  reduces  the  number  of  Barie's  cases  for  statistical  purposes  to  83. 
Schichhold,  in  1897,  added  30  to  these,  and  Vierordt,  in  1898,  brought 
the  number  of  recorded  cases  to  126.  In  1903,  Bonnet^  published  an 
article  analyzing  Barie's  findings,  and  adding  to  these  a  synopsis  of  77 
additional  cases  which  include  the  series  of  Schichhold  and  Vierordt, 
make,  together  with  the  83  cases  collected  by  Barie,  a  total  of  160, 
of  which  55  are  in  infants  and  105  in  adults.  In  addition  to  Bonnet's 
160,  the  writer^  has  collected  records  of  52  cases  not  included  by  the 

1  Revue  de  Medecine,  1886,  vi,  501.  -  Revue  de  Medecine,  1903. 

^  Of  these  52,  15  are  from  a  series  of  18  cases  collected  by  Fawcett  from  Guy's 
Hospital  Reports  and  published  in  1902;  12  are  from  the  Transactions  reported  by 
Chevers  (vol.  i,  p.  55),  Rees  (vol.  ii,  p.  203),  Peacock  (vol.  vii,  p.  83),  Lees  (vol.  xxi, 
p.  58),  Wilkes  (vol.  xi,  p.  57),  Smith  (vol.  i,  p.  52),  Barlow  (vol.  xxvii,  p.  41),  of  coarc- 
tation in  infants,  and  by  Peacock  (vol.  xii,  p.  38),  Finlay  (vol.  xxx,  p.  262),  King 
(vol.  xxiii,  p.  83),  Habershon  (vol.  xxxix,  p.  71),  Mackenzie  (vol.  xxx,  p.  66),  in  adults. 

The  remainder  are  reported  by  Preisz  (jahrb.  f.  Kinderheilk.,  xxxiii,  p.  140),  Lawrence 
and  Nabarro,  Hektoen,  Dick  {Proc.  Clin.  Path.  Society,  May  9,  1904),  Osier  {Montreal 
Gen.  Hosp.  Rep.,  P.  M.  No.  252),  Pansch,  {Giessen  Thesis,  1905)  in  infants,  and  by 
Pappenheimer  {Proceedings  New  York  Path.  Soc,  May,  1905,  January,  1906,  p.  177, 
October,  1906),  Variot,  Carmichael,  one  in  St.  Bartholomew's  Hosp.  Rep.,  vol.  i,  series 
I,  one  in  the  Museum  of  Toronto  University,  in  adults,  Monckeberg  (2  cases)  {Deut. 
Path.  Gesell.,  1907,  xi,  267),  Moon  {Lancet,  June  8,  1912),  Rokitansky  (3  cases)  {Krank 
der  Arterien,  1862,  Obs.  21),  Sella  (2  cases)  {Zieg.  Beit.,  1910,  Lxix,  501),  Strassner 
{Deut.  Arch.  f.  klin.  Med.,  1909,  hx,  349),  Wadstein,  1897  (quoted  by  Sella),  Obern- 
dorfer  {Verh.  deut.  Path.  Gesell.,  January,  1910),  MacCaUum. 


422  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

above  authors,  15  of  which  are  in  infants  and  37  in  adults,  making  a 
total  of  212  cases,  of  which  70  are  in  the  newly  born,  and  142  in  patients 
over  one  year. 

Pathogenesis. — The  proximity  of  the  stenosis  to  the  insertion  of  the 
duct  in  the  aorta  suggests  that  the  part  which  this  vessel  takes  in  the 
circulation,  or  the  changes  which  go  on  in  its  form  and  tissues  during 
its  closure  after  birth,  have  an  essential  bearing  on  the  production  of 
coarctation.  Rokitansky  (1852)  assumed  in  all  cases,  as  the  essential 
condition,  a  persistence  of  the  isthmus  and  a  consequent  weakening  of 
its  walls  so  that  they  yielded,  in  a  way  the  healthy  aorta  would  not  do, 
to  the  traction  exerted  upon  them  by  the  contraction  of  the  duct  in  its 
obliteration.  Skoda  (1855),  made  the  interesting  suggestion  that  in 
those  cases  in  which  the  isthmus  was  not  obliterated  at  birth  as  a  true 
anomaly  brought  about  by  an  atrophy  of  the  corresponding  embryonic 
aortic  arch,  the  tissue  of  the  duct  had  extended  into  the  wall  of  the  aorta, 
which  thus  contracted  as  part  of  the  same  process  by  which  the  canal 
itself  is  obliterated,  and  Brunner  (1888)  supposed  the  transplantation  of 
free  portions  of  the  ductus  tissue  into  the  adjacent  wall  of  the  aorta  to 
occur,  rather  than  its  direct  extension. 

Bonnet  gave  the  most  satisfactory  contribution  to  the  subject.  He 
divides  the  cases  of  coarctation  into  two  types,  according  as  these  occur 
in  the  newly  born  or  in  adults,  for  each  of  which  he  claims  a  different 
etiology : 

1.  The  form  described  by  him  as  that  usually  seen  in  the  newly  born 
is  a  diffuse  narrowing  of  the  isthmus,  and  is  assumed  to  be  of  develop- 
mental origin;  it  is  frequently  associated  with  grave  anomalies;  in  it 
the  ductus  arteriosus  is  often  patent.  The  cases  in  this  type  fall  again 
into  two  classes  as  regards  their  etiology:  (a)  When,  as  in  the  majority 
of  cases,  the  stenosis  is  moderate  in  degree,  it  is  explained  as  a  persist- 
ence of  the  isthmus  at  birth,  an  arrested  fetal  condition  in  which  this 
segment  fails  to  attain  its  normal  calibre,  and  the  cause  of  which  is  to 
be  sought  at  or  shortly  before  birth  in  a  simple  weakening  of  the  vessel 
wall,  the  result  probably  of  a  lowered  state  of  general  nutrition.  Thus 
Theremin  observed  that  in  the  case  of  his  so-called  normal  infant  hearts 
in  which  the  isthmus  was  abnormally  narrowed  at  birth,  there  was  a 
history  of  premature  delivery  or  of  general  weakness,  and,  conversely, 
that  in  50  per  cent,  of  infants  born  before  term  or  weakly,  marked 
narrowing  was  present,  (h)  Those  rare  cases  of  the  infantile  type,  on 
the  other  hand,  with  an  extreme  degree  of  diffuse  stenosis,  or  in  which 
the  isthmus  is  reduced  to  an  atrophic  cord,  are  probably  to  be  explained, 
as  are  also  the  few  recorded  cases  in  which  there  is  a  complete  absence 
of  the  aorta  between  the  left  subclavian  and  the  entrance  of  the  ductus, 
as  a  failure  of  development  in  early  embryonic  life  of  that  part  of  the 
fourth  left  branchial  arch  which  corresponds  to  the  isthmus  of  the  aorta. 

2.  Bonnet  places  in  a  second  class  as  the  adult  type  those  cases  seen 
usually  after  infancy  is  passed,  in  which  the  coarctation  consists  of  a 
more  or  less  abrupt  constriction  of  the  aorta  at  or  near,  often  a  little 
below,  the  insertion  of  the  ductus.  This  condition,  which  is  never  seen 
in  the  fetus,  nor  at  birth  before  the  closure  of  the  ductus  has  begun,  is. 


CONGENITAL  CARDIAC  DISEASE  423 

he  thinks,  not  of  developmental  origin,  but  is  to  be  explained  on  Skoda's 
theory  of  an  extension  of  the  peculiar  tissue  of  the  duct  into  the  adjacent 
wall  of  the  aorta,  which  thus  contracts  after  birth  along  with  the  con- 
traction of  the  arterial  canal.  As  the  malposed  tissue  is  scanty  and  tends 
to  be  of  a  width  corresponding  to  that  of  the  narrow  ductus,  its  contrac- 
tion will  have  the  effect  of  a  narrow  ligature  or  cord.  These  cases  differ 
from  those  of  the  infantile  type  not  only  in  the  character  of  the  stenosis, 
but  also  in  that  an  extensive  collateral  circulation,  giving  rise  to  marked 
physical  signs,  usually  develops,  while  serious  anomalies  are  generally 
absent,  this  last  fact  arguing  in  favor  of  its  postnatal  origin.  The  ductus 
arteriosus  may  remain  patent,  but  is  usually  obliterated. 

Associated  Anomalies. — The  distinction  drawn  by  Bonnet  between 
two  types  of  cases  offers  a  new  and  significant  suggestion.  A  statistical 
analysis,  on  the  basis  of  this  division,  of  the  212  cases  available  gives 
interesting  conJfirmation  of  this  statement,  and  points  to  a  radical  dif- 
ference in  the  etiology  of  the  two  groups.  The  following  figures  include 
as  minor  anomalies  occurring  chiefly  in  the  adult  type,  anomalous  semi- 
lunar cusps,  irregular  origin  of  the  vessels  from  the  arch,  patency  of 
the  foramen  ovale  or  duct,  persistent  left  superior  cava;  and  as  grave 
anomalies,  septal  defects,  transposition  of  the  great  trunlis,  congenital 
stenosis,  etc. 

Associated  Anomalies  in  Coarctation  in  the  Xewly  Born  (70  Cases). 

Series.  Absent.  Minor.  Grave. 

Barie 0  3  3 

Bonnet 13  11  25 

New  cases 0  3  12 

Total 13  17  40 

In  Cases  0\^r  One  Year  ("Adult  Type")  (142  Cases). 

Series.  Absent.  IMinor.  Grave. 

Barie 57  19  1 

Bonnet 15  11  2 

New  cases 8  16  8 

Total 80  46  11 

Thus  among  the  70  cases  of  stenosis  in  the  newly  born  (dying  under 
one  year),  in  only  13  instances  was  there  no  other  defect  associated; 
minor  defects  were  present  in  17  and  in  40  cases  grave  anomalies  co- 
existed. That  is  to  say,  there  is  frequently  associated  with  the  graver 
cardiac  anomalies  that  form  of  coarctation  which  may  reasonably  be 
ascribed  to  a  simple  arrest  of  development  in  later  fetal  life,  and  which 
is  due  probably  to  the  depressing  influences  that  led  to  the  associated 
defects,  or  possibly  in  some  instances  to  the  disturbed  circulation  that 
results  from  the  combined  anomaly. 

On  the  other  hand,  among  the  142  cases  in  patients  over  one  year 
(adult  type),  other  anomalies  were  absent  in  80  instances,  minor  defects 
were  present  in  46,  and  grave  anomalies  were  associated  in  only  11  cases; 
moreover,  7  of  these  11  had  not  the  characteristic  sharp  constriction 
seen  in  the  great  majority  of  these  cases,  but  were  apparently  a  persis- 


424  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

tence  of  the  infantile  type;  for  in  3  (Cbiari,  Houel,  MacKenzie)  the 
pulmonary  formed  the  descending  aorta  through  a  large  patent  duct, 
and  the  4  others  were  in  children  of  two  to  five  years  in  whom  the 
isthmus  was  simply  diffusely  narrowed.  Transposition  occurred  in  only 
1  (Fawcett),  a  child  aged  two  years  and  nine  months,  with  a  stenosis 
apparently  of  the  infantile  type.  Persistent  left  superior  cava  was  noted 
only  once  (Bonnet). 

Equally  significant  with  this  rarity  of  grave  anomalies  in  the  adult 
type  of  coarctation,  suggestive,  too,  of  some  etiological  factor  as  yet 
unknown,  is  the  frequent  association  in  this  type  of  a  certain  set  of 
minor  defects  in  the  structures  connected  with  the  aortic  arch,  namely, 
irregularities  in  the  origin  of  the  great  vessels,  absence  of  the  ductus 
(3  cases),  double  ductus  (Hammernjk),  and  especially  anomalies  of  the 
aortic  cusps,  which  last  are  relatively  so  common  as  to  seem  to  place 
their  combination  beyond  the  range  of  coincidence.  Thus  the  aortic 
valve  was  bicuspid  (in  itself  a  rare  anomaly)  in  1 5  instances;  its  segments 
were  increased  to  four  with  fusion  of  two  of  these  in  one  (Fawcett); 
in  one  instance  (Babington)  a  small  supernumerary  cusp  had  formed  on 
the  aortic  wall  above  the  others;  in  two  there  was  subaortic  stenosis, 
in  the  form  of  a  membranous  band  below  the  cusps,  and  in  one  there 
was  sub-aortic  stenosis  and  a  band  of  fibrosis  with  contraction  above 
the  aortic  cusps. 

In  the  infantile  type,  on  the  other  hand,  amid  so  many  grave  anomalies, 
bicuspid  aortic  valves  occurred  only  twice. 

Pathology. — 1.  The  diffuse  stenosis  of  the  isthmus  usually  observed 
in  infancy  and  always  present  during  the  period  of  the  fetal  circulation 
(Klotz),  is  seen  occasionally,  but  rarely,  in  later  life.  It  is  usually 
limited  below  by  the  ductus,  and  may  begin  above  as  a  gradual  diminu- 
tion of  the  arch,  or  abruptly  at  the  origin  of  the  left  subclavian  artery, 
or,  in  a  few  instances,  in  which  the  isthnms  itself  appears  to  be  placed 
higher  up  than  usual,  at  the  innominate  or  left  carotid  artery.  The 
ascending  aorta  may  be  dilated  or  of  normal  calibre,  and  below  the 
stenosis  the  vessel  may  remain  smaller  than  usual,  may  return  to  its 
full  size,  or  in  cases  where  its  descending  portion  is  supplied  by  a  patent 
duct,  be  much  dilated.  The  lining  of  the  stenosed  area  is  usually  smooth 
and  healthy.  In  degree  it  may  vary  from  a  mere  shade  below  the  normal 
to  a  lumen  of  1  to  2  mm.  in  diameter,  or  be  represented  in  rare  instances 
by  a  fibrous  obliterated  cord.  Among  the  total  212  cases,  the  pulmonary 
artery  formed  the  descending  aorta  through  a  large  patent  duct  in  15 
instances,  in  all  of  which  marked  coarctation  of  the  infantile  type  existed. 

2.  Adult  Type. — A  very  different  anatomical  character  and  a  much 
wider  variation  are  presented.  In  typical  cases  the  aorta  is  abruptly 
constricted  at  the  level  of,  or  a  little  above,  or,  most  frequently,  directly 
below  the  insertion  of  the  ductus,  as  though  by  a  tight  ligature  or  cord, 
the  groove  thus  formed  being  usually  deepest  on  the  convex  side  of  the 
arch,  which  appears  deeply  indented  as  though  cut  through  in  V-shaped 
manner  (Fig.  39).  The  aorta  on  either  side  usually  diminishes  rapidly 
toward  the  stenosis  in  an  hour-glass  or  funnel-shaped  manner,  or  it  may 
be  dilated  on  either  side,  giving  a  sausage-like  effect  (Bradley).    Viewed 


CONGENITAL  CARDIAC  DISEASE 


425 


from  within,  the  inner  surface  of  the  constriction  usually  presents  a  pro- 
jecting ridge  or  fold  corresponding  to  the  zone  of  constriction  without. 
This  may  be  so  marked  as  to  form  a  distinct  septum  bridging  across 
the  lumen,  sometimes  obliterating  it  entirely  or  leaving  a  small  central 
circular  or  triangular  lumen,  the  constriction  involving  all  the  coats  of 
the  vessel  or  only  its  inner  ones,  the  adventitia  passing  outside  of  it 
like  a  bridge.  In  other  cases  the  stenosis  may  occupy  a  wide  area  and 
appear  from  without  like  an  annular  band.  Kriejk  describes  it  in  his 
case  as  a  sort  of  resistant  ring,  enclosing  the  aorta  like  a  cuff  parallel  to 
the  axis  of  the  vessel,  and  Mannaberg  as  a  solid  segment  0.5  cm.  long 
just  below  the  insertion  of  the  duct.  The  lumen  varies  through  all 
grades  of  stenosis  down  to  one  just  admitting  a  bristle.  In  25. cases  of 
the  142  it  was  entirely  obliterated,  in  some  instances  by  a  septum  or 
diaphragm  formed  within,  but  more  frequently  by  the  elongated  annular 
form  of  constriction. 

Fig.  39 


Coarctation  of  the  aorta  in  a  woman  aged  twenty-seven  years.  Stenosis  beginning  just  beyond  the 
origin  of  the  innominate  artery;  sharp  constriction  immediately  below  the  insertion  of  the  obliterated 
ductus  {D.  A.).     (Reproduced  from  Bonnet's  article,  Revue  de  Medecine,  1903.) 


The  aorta  may  be  of  normal  calibre  above  and  below  the  stenosis,  or 
it  may  be  slightly  narrowed  at  its  origin  and  dilated  for  a  short  distance 
up.  The  diminution  in  calibre  not  infrequently  begins  at  the  innomi- 
nate or  left  subclavian  artery,  and  in  a  certain  percentage  of  cases  is 
followed  by  a  dilatation,  below  which  again  the  characteristic  tight 
constriction  near  the  duct  takes  place;  the  effect  being  that  of  a  double 
stenosis.  The  aorta  immediately  below  the  stenosis  is  often  widely 
dilated  at  the  seat  of  origin  of  the  intercostals.  Hypoplasia  of  the 
vessel  in  its  whole  length  existed  in  the  cases  of  Hale  White,  Riegel  and 
Monckeberg  (2  cases).    In  other  cases  the  aortic  walls,  otherwise  healthy, 


426 


DISEASES  OF  THE  CIRCULATORY  SYSTEM 


are  noted  as  abnormally  thin.  The  aorta  may  be  smooth  and  healthy 
in  its  whole  course,  as  in  the  cases  reported  by  Brunner  (complete  ob- 
literation), Cruveilhier,  Almagro,  Purser,  and  in  the  original  one  by 
Paris,  or  there  may  be  extensive  atheroma  with  calcification  at  the  seat 
of  stenosis,  above  or  below  it,  or  throughout  the  whole  aorta.  This 
was  present  in  39  of  the  142  cases,  in  9  of  which  it  was  definitely  stated 
to  be  at  the  seat  of  stenosis,  in  9  localized  in  the  ascending  aorta,  in  4 
localized  below  the  stenosis,  and  in  7  diffuse  throughout  the  aorta. 


Fig.  40 


Diagrammatic  representation  of  collateral  circulation  in  a  case  of  coarctation  of  the  aorta:  a,  pul- 
monary artery;  b,  arterial  duct;  c,  arch  of  aorta;  d,  coarctation  of  aorta;  e,  descending  aorta;  /,  innomi- 
nate artery;  g,  internal  mammary  artery;  h,  epigastric  artery;  i,  i,  i,  i,  deep-seated  arteries  of  neck  and 
intercostals,  forming,  together  with  the  internal  mammary  and  epigastric  arteries,  a  collateral  circulation 
with  the  thoracic  and  abdominal  aorta  and  internal  iliacs.  (From  Diseases  of  the  Heart  and  Great 
Vessels,  by  W.  H,  Walshe,  London,  1862.) 

Rupture  of  the  aorta  occurred  in  14  cases,  in  9  cases  at  the  ascending 
portion,  and  in  8  at  the  seat  of  stenosis.  Aneurism  of  the  arch  occurred 
in  11  instances,  and  in  8  of  these  it  was  of  the  dissecting  form.  Sella 
has  made  a  study  of  12  cases  of  rupture  of  the  aorta,  and  ascribes  its 
frequent  occurrence  to  the  abnormal  thinning  of  the  ascending  arch, 
which  occurs  in  many  cases  of  coarctation  and  w^hich  renders  the  coats 
more  liable  to  yield  under  the  increased  strain. 

In  most  well-marked  cases  of  coarctation  of  the  adult  type  the  blood 
supply  of  the  lower  part  of  the  body  is  maintained  by  the  development 
of  an  extensive  collateral  circulation.    The  great  \essels  of  the  arch  are 


CONGENITAL  CARDIAC  DISEASE  427 

often  enlarged  to  twice  their  calibre,  and  the  smaller  branches  involved 
are  converted  into  thick,  tortuous,  dilated  trunks.  The  principal  anas- 
tomoses are  carried  on  by  the  superior  intercostals,  the  internal  mam- 
maries,  and  the  posterior  scapular  branches  of  the  transversalis  colli 
above,  with  the  first  four  aortic  intercostals,  the  phrenic  and  superficial 
and  deep  epigastrics  beloiv  the  stenosis. 

Some  evidence  of  collateral  circulation  was  present  in  66  of  the  142 
cases  of  the  adult  type.  The  particular  branches  involved  and  the 
degree  of  dilatation  vary  greatly  even  in  cases  of  extreme  constriction. 
In  3  instances  out  of  the  142  (Barie,  Pic  and  Bonnamour,  and  Dubreuil) 
it  was  expressly  stated  to  be  absent;  in  most  of  the  remainder  the  col- 
lateral circulation  was  not  mentioned,  but  this  does  not  imply  that  it 
was  always  absent  entirely,  as  minor  alterations  in  the  peripheral  vessels 
are  easily  overlooked. 

The  ductus  arteriosus  was  patent  in  13  of  the  142  adult  cases.  In  some 
instances  the  ligamentum  arteriosum  is  described  as  "solidified"  or 
much  thickened  as  though  by  inflammatory  action.  Among  the  70 
cases  in  infants  under  one  year  the  ductus  was  patent  51  times. 

Marked  hypertrophy  with  dilatation  of  the  heart  is  rare  in  infants,  but 
occurs  in  the  majority  of  the  older  cases,  being  noted  in  87  out  of  the 
142.  It  is  stated  by  most  authors  to  be  the  direct  result  of  the  obstruc- 
tion in  the  course  of  the  descending  arch,  but  the  relation  of  the  two 
conditions  is  rendered  uncertain  by  the  frequent  association  of  chronic 
valvular  disease,  which  must  be  a  factor  in  the  hypertrophy.  More- 
over, a  few  cases  are  recorded  {e.  g.,  Reynaud's  aged  ninety-two,)  in 
which,  with  marked  constriction  or,  even,  as  in  Brunner's  case,  s0 
obliteration  at  the  insertion  of  the  duct,  the  heart  has  remained  norrhal 
throughout  life.  This  w^as  stated  to  be  the  case  in  10  of  the  142  cases. 
Although  this  is  a  small  percentage,  it  proves  that  new  channels  provided 
for  the  blood  by  the  dilated  collaterals  may  be  sufficient  to  carry  on  the 
circulation  without  increasing  the  work  of  the  heart.  In  Dumontpallier's 
patient,  aged  thirty-nine  years,  in  whom  the  stenosis  was  produced  by 
a  septum  with  triangular  central  opening,  13  mm.  in  diameter,  and  "the 
heart  was  not  hypertrophied,  the  collateral  circulation  was  carried  on 
chiefly  by  the  aortic  intercostals  and  the  vessels  from  the  subclavian, 
the  anastomosis  between  the  internal  mammary  and  the  epigastrics 
being  little  developed. 

On  the  other  hand,  an  analysis  of  the  87  cases  with  hypertrophy 
shows  that  while  50  w^ere  complicated  with  chronic  valvular  lesions  or 
other  cardiac  defects,  in  the  remaining  38  no  cause  was  present  except 
the  coarctation  itself.  Of  the  latter,  there  were  38  cases  of  hypertrophy 
without  any  assignable  cause  except  the  coarctation,  hypertrophy  was 
confined  to  the  left  ventricle  in  9,  and  involved  the  whole  heart  in  30, 
of  which  latter  the  left  ventricle  especially  w^as  enlarged  in  9.  An  in- 
teresting point  is  that  in  20  of  the  38,  the  collateral  circulation  was 
either  stated  to  be  absent  or  was  not  mentioned,  and  in  6  more  it  was 
not  much  developed,  the  onFy  sign  noted  being  a  dilatation  of  the  great 
vessels  of  the  arch.  In  the  12  remaining  cases  a  collateral  circulation 
had  developed,  but  in  4  of  these  the  cardiac  hypertrophy  was  only  slight. 


428  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

These  facts  argue  that  even  in  extreme  degrees  of  constriction  the  heart 
may  remain  normal  in  the  presence  of  an  adequate  collateral  circulation, 
but  that  when  this  becomes  insuflBcient,  cardiac  hypertrophy  and  dila- 
tation supervene. 

Age  and  Sex. — A  remarkable  predominance  of  the  adult  type  of  the 
anomaly  in  the  male  sex  is  noted  by  all  writers.  Among  133  of  the  142 
cases  over  one  year,  92  were  in  males,  and  41  in  females. 

That  the  stenosis  does  not  necessarily  interfere  with  the  duration  of 
life  is  proved  by  the  fact  that  9  patients  died  in  the  sixth  and  9  in  the 
seventh  decade,  while  one  (Reynaud's)  lived  to  the  age  of  92.  More 
than  half  of  the  remainder,  however  (63  cases),  died  between  the  ages 
of  twenty  and  forty  years;  indicating  that,  in 'the  anomalous  conditions 
of  the  circulation  that  prevail,  the  system  is  not,  as  a  rule,  equal  to 
the  full  demands  of  the  stress  of  normal  existence. 

In  the  142  cases  over  one  year  death  occurred  as  follows : 

Cases. 

1  to    5  years 7 

5  to  10      "        4 

10  to  15      "        4 

15  to  20      "        17 

20  to  30     "        37 

30  to  40      "        26 

40  to  50      "        16 

50  to  60      "        9 

60  to  70      " 9 

Over  70      "        1 

Adults  (exact  age  not  mentioned) 12 

Symptoms  and  Course. — Coarctation  in  infants  is  of  little  clinical 
significance,  except  in  so  far  as  it  may  complicate  other  grave  anomalies. 
In  the  adult  type  it  is  a  condition  of  the  greatest  interest  and  impor- 
tance. Symptoms  when  present  may  be  distinguished  as  those  associated 
with  the  lesion  and  those  of  the  cardiac  insufficiency  which  frequently 
supervenes.  As  characteristic  of  the  overtaxing  of  the  altered  circula- 
tion, in  which  the  blood  supply  to  the  head  and  upper  extremities  is 
freer  than  that  to  the  lower  part  of  the  body,  may  be  mentioned;  violent 
pulsations  (Flaherty's  case),  plethora  with  sleeplessness  and  continuous 
buzzing  in  the  ears  (Legrand),  violent  headaches  (Hammernjk),  lividity 
of  the  face  (Che vers.  Purser,  Kjellberg),  suffusion  of  the  head  and  neck 
(Moore),  epistaxis  and  hemoptysis  (Flint);  in  Dubreuil's  case,  a  vas- 
cular surcharge  of  the  head  and  chest  contrasted  with  an  atony  of  the 
subdiaphragmatic  viscera;  in  that  of  Redenbacher,  a  boy  aged  seven 
years,  with  a  stenosis  at  the  isthmus  admitting  a  crow-quill,  and  exten- 
sive collateral  circulation,  the  development  of  the  head  and  upper  ex- 
tremities was  in  advance  of  that  of  the  lower  part  of  the  body.  Severe 
thoracic,  epigastric,  or  abdominal  pain  and  vomiting  of  long  standing 
(Roemer's  case),  or  pains  in  the  back  or  lower  extremities  (Lebert) 
occur,  and  may  perhaps  be  due  to  the  local  effect  of  the  constipation. 
Of  significance  is  Muriel's  report  of  a  man  aged  twenty-five  years,  who 
was  always  weakly,  and  who  developed  severe  pains  in  the  back  and 
symptoms  of  aneurism  of  one  of  the  large  vessels  of  the  chest;  post- 


CONGENITAL  CARDIAC  DISEASE  429 

mortem  a  dense  mass  of  enlarged  glands  the  size  of  a  hen's  egg  was 
found  adherent  to  the  aorta  at  the  point  of  its  constriction;  it  had  eroded 
the  dorsal  vertebree.  Precordial  pain  and  oppression,  dyspnoea,  and 
severe  palpitation  indicate  the  cardiac  strain.  Cyanosis  is  extremely 
rare  except  as  a  terminal  event;  in  only  2  uncomplicated  cases  in  the 
whole  series  (Almagro  and  Carmichael),  in  both  of  which  the  ductus 
was  patent,  was  a  true  congenital  cyanosis  present  throughout  life. 
liack  of  development  was  noted  three  times,  delayed  menses  once. 
Many  end  with  a  stage  of  failing  compensation,  which,  in  those  not 
complicated  by  chronic  valvular  disease,  is  usually  identical  with  that 
of  mitral  incompetence. 

Symptoms  are  (a)  absent,  (b)  late  in  developing,  or  (c)  present  through- 
out life,  (a)  In  some  of  the  most  w^ell-marked  cases  of  constriction 
or  even  obliteration  at  the  isthmus,  symptoms  are  absent  throughout 
life.  The  condition  may  be  quite  latent,  and  constitute,  in  Barie's 
words,  a  "surprise  d'aviphitheatre"  at  the  autopsy,  death  occurring  from 
some  intercurrent,  independent  disease.  Thus  Crisp  describes  a  chance 
finding  of  a  stenosis  admitting  a  goose-quill  in  a  soldier  aged  fort^'-eight 
years,  who  had  been  in  excellent  health  and  had  died  accidentally,  and 
Scheiber  complete  obliteration  of  the  descending  aorta  in  a  man  aged 
forty-one  years,  dying  of  pneumonia,  who  showed  no  signs  of  heart 
disease.  In  these  latent  cases  sudden  death  may  occur.  In  most 
instances  a  rupture  of  the  heart  or  aorta  is  found  (Liittich,  Barker, 
Wise,  Legg,  and  others).  Death  took  place  without  previous  warning 
or  symptoms  in  16  of  the  142  cases,  constituting  an  event  of  sufficient 
frequency  to  have  an  important  medicolegal  bearing. 

(b)  A  large  proportion  of  the  cases  are  in  able-bodied,  vigorous  men, 
in  w^hom  the  lesion  long  remains  latent,  symptoms  developing  as  the 
altered  circulation  becomes  overtaxed,  or  on  the  intercurrence  of  some 
complicating  condition,  especially  endocarditis.  Not  infrequently, 
symptoms  developing  late  in  life  are  entirely  cardiac  in  character,  cases 
otherwise  latent  terminating  with  a  stage  of  failing  compensation  which 
may  be  due  to  the  lesion  itself,  or  to  the  chronic  valvular  disease  so  often 
associated. 

(c)  In  a  few  instances  only,  symptoms  of  some  obstruction  in  the 
cardiovascular  system  are  present  throughout  life.  Quinquaud's  patient, 
a  youth  aged  nineteen  years,  sufl'ered  from  infancy  with  intense  palpi- 
tation and  violent  dyspnoea,  so  that  he  could  not  join  in  play,  and 
oedema  of  the  extremities  developed  shortly  before  death,  which  occurred 
suddenly.  Erman's  patient  was  weakly  and  had  always  sufi^ered  from 
dyspnoea.  Death  took  place  at  19,  after  seven  and  a  half  months  of 
failing  compensation.  Lebert's  patient,  aged  twenty-two  years,  had 
long  had  epistaxis  and  dyspnoea,  and  developed  cardiac  symptoms  in  the 
last  two  years. 

Physical  Signs. — These  bear  no  constant  relation  to  the  symptoms, 
but  may  be  present  where  these  are  quite  lacking.  Nor,  on  the- other 
hand,  do  they  correspond  to  the  degree  of  the  constriction,  nor  to  the 
extent  of  the  collateral  circulation,  both  of  which  may  be  developed  to 
an  extreme  degree  without  yielding  any  evidence  of  their  presence. 


430  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

The  most  marked  signs  appear  to  be  produced  in  association  with  chronic 
valvular  disease  or  bicuspid  aortic  vaKes,  or  with  the  relative  mitral 
incompetency  of  the  later  stages  of  the  cardiac  dilatation  that  frequently 
supervenes,  in  which  cases  the  murmurs  formed  in  the  heart  may  be 
propagated  along  the  vessels.  The  signs  peculiar  to  the  lesion  may  best 
be  studied  in  uncomplicated  cases.  They  are  both  vascular  and  cardiac, 
and  are  present  in  varying  degrees  and  combinations  in  the  majority 
of  cases. 

Vascular. — These  depend  chiefly  upon  the  inequality  of  the  circula- 
tion in  the  upper  and  lower  halves  of  the  body,  and  upon  the  unusual 
appearances  presented  b}^  the  dilated  collaterals.  In  well-marked  cases 
the  vessels  of  the  upper  half  of  the  trunk  may  be  seen  pulsating,  the 
subclavians,  as  a  rule,  more  markedly  than  the  carotids;  and  pulsation 
may  be  traced  in  many  cases  along  the  abnormally  dilated  and  tortuous 
vessels  occupying  the  course  of  the  internal  mammaries  on  either  side 
of  the  sternum,  or  the  posterior  intercostal  or  scapular  arteries  behind. 
In  Libman's  patient  there  was  a  varicose  mass  beneath  the  skin  of  the 
abdomen;  in  Flint's,  both  supraspinous  fossse  were  occupied  by  a  net- 
work of  tortuous  pulsating  vessels;  in  Leudet's,  small  arterial  dilatations 
extended  over  the  middle  of  the  thorax  both  in  front  and  behind,  and 
were  most  marked  at  the  posterior  border  of  the  left  axilla  and  in  the 
left  supraspinous  fossa,  where  they  formed  tortuous,  thickened  vessels, 
pulsating  synchronously  with  the  radials.  Along  the  whole  course  of 
these  a  murmur,  usually  postsystolic  in  rhythm,  but  sometimes  systolic 
or  double,  may  be  heard,  and  a  slight  thrill  may  be  felt. 

The  radial  pulse  is  frequently  hard  and  full,  and  may  be  unequal  on 
the  two  sides.  The  lower  extremities  may  contrast  strangely  with  the 
upper  half  of  the  body  in  the  absence  of  all  visible  pulsations.  On 
examination  the  pulse  in  the  abdominal  aorta  and  femorals  is  either 
very  weak  or  absent,  while  the  murmur  usually  audible  on  pressing  over 
the  femoral  with  the  stethoscope  cannot  be  heard.  In  Bonnet's  case, 
diagnosed  before  death,  no  pulse  could  be  felt  in  the  abdominal  aorta 
or  femorals,  and  an  artery  pulsating  visibly  and  as  large  as  the  radial, 
over  which  an  intense  systolic  murmur  could  be  heard,  ran  downward 
between  the  vertebral  column  and  the  inner  border  of  the  left  scapula. 
On  the  right  side  of  the  column  a  similar  but  less  strong  pulsation  could 
be  felt,  but  no  murmur  was  heard. 

Hornung's  patient,  a  man  aged  twenty-seven  years,  is  an  example  of 
an  extreme  stenosis  not  producing  any  symptoms,  but  with  marked 
physical  signs,  in  whom  death  occurred  suddenly  from  rupture  of  the 
aorta.  There  was  energetic  pulsation  and  a  systolic  murmur  over  the 
carotids  and  subclavians.  At  the  inner  border  of  the  scapular  region 
were  sinuous  pulsating  vessels.  The  radial  pulse  was  hard  and  resistant, 
and  there  was  no  pulsation  in  the  abdominal  aorta,  popliteral,  posterior 
tibial,  or  pedal  arteries.  As  long  ago  as  1839,  Mercier  diagnosed  a  case 
in  which  there  were  visible  pulsations  in  the  intercostals,  a  marked  bruit 
at  the  lower  angle  of  the  left  scapula,  and  a  very  weak  pulse  in  the  lower 
extremities,  with  epistaxis  and  symptoms  of  failing  compensation  for 
three  months  before  death. 


CONGENITAL  CARDIAC  DISEASE  431 

Cardiac. — The  heart's  action  may  be  tumultuous,  with  a  heaving  im- 
pulse, and  the  organ  may  present  evidences  of  enlargement,  particularly 
of  the  left  ventricle.  A  precordial  thrill  was  present  in  only  3  of  the 
cases.  The  heart  sounds  may  be  quite  pure,  or  accompanied  by  loud 
murmurs,  usually  systolic  or  postsystolic  in  rhythm.  In  Hornung's 
patient  a  rough  murmur  was  heard  at  the  aortic  area,  most  marked 
between  the  left  clavicle  and  the  third  rib.  In  Decker's,  a  woman  aged 
nineteen  years,  with  complete  obliteration  at  the  isthmus  and  no  com- 
plicating valvular  dis'^ase,  a  rasping  murmur  filling  the  whole  systole 
was  heard  at  the  apex,  and  could  also  be  traced  along  the  thickened, 
tortuous,  and  dilated  arteries,  among  which  the  superior  epigastric,  the 
long  thoracic,  and  the  dorsalis  scapulae  formed  pulsating  cords;  the  heart 
was  hypertrophied. 

Diagnosis. — When  such  symptoms  and  signs  as  the  above  occur 
together  a  very  distinctive  clinical  picture  may  be  formed.  It  must  be 
remembered  that  they  may  be  entirely  absent,  or  present  only  in  a  frag- 
mentary way,  such  as  may  awaken  suspicion  of  the  reality,  yet  render 
a  positive  diagnosis  impossible.  The  fact  that  physical  signs  as  well  as 
symptoms  usually  do  not  remain  stationary,  but  progress  to  a  more  defi- 
nite development,  furnishes  the  clue  by  which  the  presence  of  the  anomaly 
may  best  be  traced.  A  pulsation  at  an  abnormal  area,  or  a  superficial 
murmur  of  unusual  site,  noted  and  watched,  may  lead  to  a  second  exami- 
nation, at  which  the  full  development  of  the  condition  may  be  revealed. 

Even  where  symptoms  are  present,  the  diagnosis  may  be  very  diffi- 
cult between  a  constriction  of  the  descending  aorta  at  the  isthmus  and 
obstruction  of  this  vessel  or  its  branches  by  aneurism,  or  by  the  pressure 
of  a  mediastinal  tumor.  The  absence  of  any  considerable  area  of  dulness, 
the  transmission  of  the  murmur  for  long  distances  along  the  branches 
of  the  ascending  arch,  the  remarkable  extent  to  which  the  collateral 
circulation  is  sometimes  developed,  above  all,  the  results  of  a-ray  exami- 
nation, contribute  differential  points  in  favor  of  coarctation.  In  perhaps 
no  other  pathological  condition  are  more  extensive  changes  compatible 
with  fewer  evidences  during  life.  The  later  stage  of  cases  in  which  vascular 
phenomena  are  lacking  and  failing  compensation  develops,  may  be  impos- 
sible to  distinguish  from  that  of  organic  insufficiency  of  the  mitral  valve. 

Termination. — The  cases  may  be  divided  into  three  groups:  (1)  The 
condition  may  be  latent  throughout  life  and  not  interfere  with  its  dura- 
tion in  any  way.  (2)  Both  in  latent  cases  and  in  those  presenting  symp- 
toms during  life  death  may  occur  suddenly,  by  asystole,  from  rupture 
of  the  heart  or  aorta,  or  from  causes  unknown.  (3)  Death  may  follow  a 
stage  of  broken  compensation,  which  may  be  preceded  by  symptoms 
characteristic  of  the  lesion,  or  may  develop  suddenly  in  an  apparently 
healthy  subject. 

HYPOPLASIA  OF  THE  AORTA  AND  ITS  BRANCHES. 

Hypoplasia  of  the  aortic  system  may  be  described  as  that  condition  in 
which  the  lumen  of  the  arterial  vessels  in  the  greater  circulation  remains 
abnormally  small  and  the  walls  unnaturally  thin  and  elastic.    The  heart 


432  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

may  also  be  reduced  in  size  or  may  undergo  a  compensatory  dilatation 
and  hypertrophy  which  involves  especially  the  left  ventricle,  but  may 
extend  to  the  whole  organ,  and  is  usually  succeeded  by  a  marked  degree 
of  secondary  dilatation.  The  subjects  are,  as  a  rule,  pale  individuals  of 
delicate  frame,  who  present  signs  of  retarded  development,  such  as  a 
delayed  advent  of  the  signs  of  puberty.  Anomalies  of  the  sexual  organs 
frequently  occur.  The  general  health  is  usually  fair  until  early  adoles- 
cence, when  the  condition  generally  manifests  itself  after  some  unusual 
physical  strain  has  been  endured,  by  the  sudden  appearance  of  failing 
compensation.  The  course  is  then  progressively  downward.  In  women, 
who  are  by  natural  conditions  less  exposed  to  undue  muscular  exertion 
than  are  men,  this  stage  of  cardiac  insufficiency  may  not  supervene,  but 
the  disease  may  run  its  course  under  the  guise  of  a  chlorosis.  By  some 
observers  (Ortner,  Hiller)  the  narrowing  of  the  vessels  is  thought  to  pre- 
dispose to  the  infectious  fevers,  and  a  special  group  of  cases  in  which 
death  has  occurred  from  typhoid  fever  is  described.  It  is  also  seen  in 
young  anemic  subjects  dying  of  pulmonary  tuberculosis. 

There  has  been  some  debate  as  to  the  pathological  significance  of  the 
condition.  Several  authors  have  maintained  that  the  greater  elasticity 
of  the  walls  of  the  vessels  compensates  for  their  smaller  calibre,  and  so 
prevents  undue  strain  upon  the  heart.  A  number  of  statistical  contribu- 
tions, have,  however,  demonstrated  that  hypoplasia  of  the  aorta  must 
be  given  a  place  in  pathogenesis  as  one  of  the  special  causes  of  cardiac 
asystole.  The  etiology  is  obscure.  In  some  few  cases,  such  as  the 
cachexias  of  wasting  diseases,  a  true  atrophy  of  the  aorta  occurs.  In 
the  majority  some  congenital  defect,  amounting  in  some  instances  to 
a  congenital  tendency  to  dwarfism,  may  be  supposed.  This  view  is 
supported  by  the  frequent  association  of  other  anomalies,  especially  in 
the  generative  and  circulatory  systems. 

Typical  cases  were  described  by  Morgagni  in  1761  and  by  Meckel  in 
1788.  Rokitansky  defined  the  condition  in  1838  and  commented  upon 
its  association  in  some  instances  with  defects  of  the  external  genitalia. 
Bamberger,  in  1843,  noted  the  association  of  chlorosis  with  a  small 
aorta.  But  in  general  the  subject  attracted  little  attention  until  Virchow, 
in  1872,  published  a  series  of  cases  illustrating  the  frequency  of  a  small 
elastic  aorta  and  a  small  heart  in  chlorosis,  and  suggested  an  etiological 
relation  between  the  two  conditions.-  He  explained  the  absence  of  com- 
pensatory hypertrophy  of  the  heart  in  some  cases  and  its  presence  in 
others,  as  depending  upon  the  degree  of  diminution  of  the  lumen  of  the 
vessels,  the  volume  of  the  circulating  blood,  the  elasticity  of  the  vessel 
wall,  and  the  amount  of  work  done  by  the  individual.  Ortner,  in  1891, 
dwelt  chiefly  on  the  medicolegal  aspect  of  the  subject,  and  emphasized 
as  pathognomonic  an  absence  of  the  jugular  pulsation  in  the  episternal 
notch  in  cases  in  which  the  upper  border  of  the  cardiac  dulness  is  high. 
Spitzer  (1897)  attempted  by  a  study  of  the  recorded  material  to  place 
the  condition  on  a  more  definite  clinical  basis.  He  pointed  out  that 
while  the  cases  usually  terminate  with  failing  compensation,  this  resem- 
bles the  end  stages  of  chronic  valvular  disease  only  in  a  general  way, 
that  the  symptoms  are  in  general  those  of  a  cardiac  overstrain  due  to 


CONGENITAL  CARDIAC  DISEASE  433 

muscular  fatigue,  and  have  a  progressive  tendency  to  grow  worse;  that 
during  the  stage  of  broken  compensation  the  cardiac  dulness  is  usually 
much  enlarged,  and  that  the  sounds  are  generally  clear,  with  marked 
pulmonary  accentuation,  although  occasionally  accompanied  by  mur- 
murs. Like  Virchow,  he  noted  as  characteristic  a  remarkable  pallor, 
but  he  ascribed  it  not  to  a  diminution  of  the  hemoglobin,  which  he  found 
usually  90  to  100  per  cent.,  but  to  the  reduction  in  size  of  the  vessels 
through  which  a  smaller  quantity  of  blood  coursed  beneath  the  skin. 

Burke^  (1901)  gave  a  historical  review  of  the  subject  and  a  full  account 
of  all  the  cases  on  record.    He  divided  the  material  into  four  groups: 

(1)  Hypoplasia  of  the  aorta  in  the  so-called  blood  diseases,  as  chlorosis, 
pernicious  anemia,  hemophilia;  (2)  hypoplasia  in  association  with  infec- 
tious diseases,  considered  as  predisposing  to  these  or  tending  to  their 
fatal  termination;  (3)  hj^Doplasia  with  general  dystrophies,  as  acromegaly; 
(4)  hypoplasia  presenting  the  picture  of  a  cardiac  lesion,  the  mass  of  the 
cases  belonging  to  this  last  group.  Apelt^  collected  100  cases  from  the 
literature  and  added  an  account  of  two  cases,  both  of  which  were  diag- 
nosed during  life.  The  subjects  were  young  men  aged  seventeen  and 
twenty-one  years,  of  slight  build  and  medium  size,  who  had  been  capable 
of  the  usual  amount  of  physical  exertion,  and  had  presented  no  symptom 
of  disease.  Both  passed  through  a  period  of  unusual  physical  strain 
just  before  the  sudden  onset  of  symptoms,  which  took  place  a  few  weeks 
before  death.  The  picture  was  that  of  an  acute  dilatation  of  the  heart 
with  slight  terminal  cyanosis,  oedema,  ascites,  the  cardiac  area  enormously 
increased,  and  the  pulmonary  second  sound  markedly  accentuated. 
The  heart  sounds  were  pure  except  toward  the  close  in  one  patient,  in 
whom  a  systolic  mitral  murmur  developed.  Postmortem,  in  both  cases, 
the  arteries  were  throughout  thin,  delicate,  elastic,  and  of  diminished 
calibre,  and  there  was  moderate  hypertrophy  with  great  pathological 
dilatation  of  the  heart,  although  its  valves  and  chordae  tendinese  were 
delicate,  and  healthy.  Microscopic  examination  revealed  an  entire 
absence  of  fatty  degeneration  of  the  myocardium. 

Van  Ritook  analyzed  73  cases,  56  from  the  literature  including  the 
series  of  Burke  and  Apelt,  and  17  from  personal  observation,  and  he 
enumerated  the  following  points  as  of  diagnostic  value:  (1)  The  youth 
of  the  patient.  (2)  Marked  and  obstinate  anemia  persisting  in  spite 
of  all  treatment.  (3)  The  early  development  of  fatigue  in  a  young  indi- 
vidual on  slight  physical  exertion.  (4)  Subnormal  temperature  or  only 
slight  rise  of  temperature  in  febrile  diseases.  (5)  Palpitation.  (6)  Hyper- 
trophy of  the  left  heart.  (7)  Acute  cardiac  insufficiency  developing 
after  comparatively  slight  physical  strain.  (8)  Diminished  resistance  to 
infectious  diseases. 

ANOMALIES    OF    THE   AORTIC   ARCH. 

Quite  a  wide  variation  of  anomalous  conditions  of  the  aortic  arch  and 
its  branches  occur,  the  individual  forms  of  which  repeat  themselves 

^Deut.  Arch.f.  Uin.  Med.,  1901,  No.  71,  187. 
2  Deut.  med.  Woch.,  1905,  xxxi,  1186. 
VOL.  IV — 28 


434  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

in  different  subjects  with  such  similarity,  that  an  underlying  develop- 
mental error  may  be  inferred.  The  units  of  the  series  may  be  summed  up 
under  the  various  headings  of  (1)  double  aortic  arch,  (2)  right  aortic 
arch,  (3)  origin  of  left  subclavian  artery,  from  (a)  a  patent  ductus  arterio- 
sus, or  (b)  the  pulmonary  artery,  (4)  origin  of  the  right  subclavian 
artery  from  the  descending  thoracic  aorta  below  the  left  subclavian 
artery,  and  (5)  common  brachiocephalic  trunk.  In  all  these  the  under- 
lying defect  is  either  a  persistence  of  an  embryonic  arch  which  normally 
undergoes  involution  (double  aortic  arch,  right  aortic  arch,  left  subclavian 
from  patent  ductus),  or  an  arrested  development  of  a  portion  of  the 
embryonic  arches  that  normally  persists  (right  subclavian  from  thoracic 
aorta,  common  brachiocephalic  trunk).  In  man  the  primitive  aorta 
is  at  first  double  and  of  the  six  embryonic  arches  the  first,  second,  and 
fifth  disappear  on  both  sides  as  w^ell  as  the  left  sixth  and  the  distal 
part  of  the  left  fourth,  while  the  third  parts  persist  as  the  carotid 
arteries,  the  fourth  left  as  the  aorta,  the  proximal  portion  of  the  right 
fourth,  as  the  subclavian,  and  the  left  sixth  becomes  the  pulmonary 
artery  with  the  ductus  arteriosus. 

Double  Aortic  Arch. — In  this  anomaly,  of  which  some  8  cases  have 
been  recorded,  the  aorta  ascends  to  the  right  and  turns  backward  and 
divides  near  the  beginning  of  its  transverse  portion  into  two  large  trunks 
which  lie  parallel  with  each  other  and  unite  just  beyond  the  insertion 
of  the  ductus  to  form  the  descending  arch,  enclosing  between  them  an 
elliptical  space  in  which  the  oesophagus  and  trachea  lie  embraced  within 
the  vascular  circle  thus  produced.  The  posterior  member  of  the  pair, 
which  is  usually  the  larger,  appearing  as  the  true  arch  of  the  aorta,  gives 
off  the  right  carotid  and  subclavian,  and  lies  behind  the  trachea.  The 
smaller  anterior  limb  lies  below  the  other,  appearing  like  a  loop  from  it, 
and  gives  off  the  left  carotid  and  subclavian,  either  as  a  simple  trunk 
(left  innominate)  or  as  separate  vessels.  Examples  are  the  cases  recorded 
by  Curnow^  in  a  woman,  aged  eighty-seven  years,  and  by  Hamdi,^ 
in  a  woman  aged  forty-five  years.  In  the  latter  case  the  trachea  and 
oesophagus  were  slightly  compressed.  Although  no  symptoms  had  been 
produced,  the  deformity  of  the  trachea  was  sufficient  to  prove  the  possi- 
bility of  a  fatal  obstruction. 

Henle  explains  the  posterior  limb  of  the  double  aorta  as  a  persistence 
of  the  fourth  right  arch.  The  anterior  limb  represents  the  fourth  left 
arch,  and  the  two  unite  at  the  point  of  insertion  of  the  ductus  (sixth 
arch)  to  form  the  descending  aorta  as  in  the  embryo,  and  as  is  persistent 
in  the  amphibia. 

Right  Aortic  Arch. — In  this  anomaly  the  aorta  is  normal  at  its  origin, 
but  curves  over  the  root  of  the  left  instead  of  the  right  lung,  so  that 
its  convexity  lies  to  the  left,  and  it  passes  down  on  the  right  side  of  the 
aorta,  the  right  recurrent  laryngeal  nerve  hooking  round  the  arch  in 
the  same  manner  as  does  the  left  under  normal  conditions.  The  left 
carotid,  or,  in  some  cases,  a  left  innominate  artery,  arises  from  the  front 
of  the  aorta  shortly  after  its  origin  and  represents  the  persistent  left 

1  Trans.  Path.  Soc,  London,  1874,  xxvi,  23. 

2  Deut,  wed.  WocK,  1906,  xxxii,  1410. 


CONGENITAL  CARDIAC  DISEASE 


435 


aortic  root.  The  right  carotid  rises  next  in  about  its  normal  situation, 
and  then  the  right  subclavian  more  posteriorly  and  to  the  right,  while 
the  left  subclavian  arises  either  (a)  in  its  normal  situation  or  (6)  with 
the  left  carotid  from  the  left  innominate,  or  from  a  patent  ductus  or  from 
the  pulmonary  artery. 

Fig.  41 

. .  -  Car.In  t.  Sin  is  tree 


Car.lnt.  D extra  ■  - 
Car.Ext.Bextra 

Art.  Car.Comi7i.Dextra 
ArcAortoE.  Dextra 


Aorta  D esc 


CarExtSinistra 


Art.Car.  Co7fi?n.  Si7tistra 
Art.Suicl.  Simstiu 


Zi^mientuTn  Arteriosiim 
Art.Pulm.  Si?iistra 


Dr.  Kaufmann's  case  of  right  aortic  arch  with  ligamentum  arteriosum  encircling  the  trachea  and 
oesophagus.  Diagrammatic  representation  following  Evans'  diagram  of  the  survival  of  the  aortic  arches, 
to  show  that  in  the  present  case  the  fourth  right  arch  (represented  by  the  arch  and  trunk  of  the  descend- 
ing thoracic  aorta),  the  left  proximal  part  of  the  fourth  left  arch  (represented  by  the  left  innominate 
and  subclavian),  and  the  left  sixth  arch  (represented  by  the  hgamentum  arteriosum),  survive,  and  that 
the  trachea  is  necessarily  encircled  by  the  passing  over  of  the  ductus  to  the  arch  of  the  opposite  side. 
The  oesophagus  is  here  omitted  for  the  sake  of  clearness:  Car.  Int.  Sinistra,  left  internal  carotid;  Car. 
Ext.  Sinistra,  left  external  carotid;  Art.  Car.  Comm.  Sinistra,  left  common  carotid;  Car.  Int.  Dextra, 
right  internal  carotid;  Car.  Ext.  Dextra,  right  external  carotid;  Art.  Car.  Comm.  Dextra,  right 
common  carotid;  Art.  Subcl.  Sinistra,  left  subclavian;  Art.  Subcl.  Dextra,  right  subclavian;  Arc. 
Aortic  Dextra,  right  aortic  arch;  Art.  Pulm.  Sinistra  and  Dextra,  left  and  right  pulmonary  arteries; 
D.  A.,  funnel-sbaped  patent  aortic  end  of  ductus;  A.  P.,  pulmonary  arterj'.  (Drawing  by  Prof.  G. 
Adami,  McGiU  University.) 

The  curve  of  the  aorta  passing  from  the  right  to  left  and  then  back 
to  the  right  side  of  the  vertebral  column  becomes  very  sinuous  in  those 
cases  in  which  the  ligamentum  arteriosus  or  a  patent  ductus  remains 
attached  to  the  right  aortic  arch  at  its  usual  site  of  insertion  opposite 


436  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

or  near  the  left  subclavian.  In  these  cases  the  ductus  is  forced  to  pass 
from  its  origin  in  the  pulmonary  artery,  on  the  left  side  anteriorly,  back- 
ward and  to  the  right  to  meet  the  right  arch  which  curves  toward  it 
beJiind  the  trachea  and  oesophagus  which  are  thus  again,  as  in  double 
aortic  arch,  engaged  in  a  complete  vascular  circle  formed  in  this  case 
by  the  aorta,  ductus  arteriosus  (patent  or  obliterated),  and  the  pul- 
monary artery.  Four  such  cases  are  recorded,  in  two  of  which  the 
ductus  was  obliterated;  in  the  third,  it  was  widely  patent,  and  in  the 
fourth  case  (a  specimen  in  the  McGill  Museum  presented  by  Dr.  Kauf- 
mann,  which  has  been  figured  for  me  diagrammatically  by  Professor 
Adami,  see  Fig.  41),  it  was  widely  patent  at  its  aortic  end,  but  was 
closed  beyond,  the  ligamentum  arteriosum  forming  a  long  thick  cord. 
In  the  latter  case  the  aorta  gave  off  a  left  innominate  trunk  and  then 
curved  backward  soon  after  its  origin  and  to  the  left,  passing  behind  the 
trachea  and  oesophagus  and  gaining  the  right  side  of  the  vertebral 
column  below.  At  the  point  where  the  convexity  of  the  arch  gains  the 
left  side  of  the  trachea,  it  presents  a  deep  triangular  pouch,  which  repre- 
sents the  patent  aortic  end  of  the  ductus,  to  the  apex  of  which  externally 
a  cordlike  structure  of  remarkable  length  and  thickness,  the  ligamentum 
arteriosum,  is  attached.  This  ligament  passed  forward  anteriorly  to  the 
trachea  and  oesophagus  to  its  attachment  in  the  left  branch  of  the  pul- 
monary artery,  and  encloses  these  viscera  within  the  vascular  circle 
formed  by  it  with  the  aorta  and  pulmonary  artery. 

Such  cases  form  a  link  between  simple  right  aortic  arch,  in  which  the 
aorta  lies  entirely  on  the  right  side  of  the  trachea,  and  double  aortic 
arch  in  which  trachea  and  oesophagus  are  completely  embraced  by  a 
vascular  ring.  They  throw  light  on  the  development  of  the  latter,  at 
first  sight  inexplicable,  phenomenon.  For,  since  a  right  aortic  arch  must 
pass  behind  the  trachea  to  unite  with  the  persistent  sixth  left  arch  which 
is  represented  by  the  ductus,  it  must  do  the  same  to  unite  with  persistent 
left  fourth  arch  which  is  represented  by  the  anterior  limb  of  the  double 
pair.  The  whole  situation  is  explained  by  the  reflection  that  in  the 
embryo  these  viscera  occupy  a  position,  not  behind,  but  on  the  left 
posterior  aspect  of  the  primitive  heart,  and  that  the  pairs  of  embryonic 
arches  pass  on  either  side  of  them  to  their  destination  in  the  dorsal  aorta, 
so  that  if  arches  on  opposite  sides  unite  as  they  have  done  in  the  ano- 
malies under  consideration,  trachea  and  oesophagus  are  bound  to  be 
encircled. 

Left  Subclavian  from  Ductus  Arteriosus  or  Pulmonary  Artery. — 
While  the  left  subclavian  is  given  off  from  the  fourth  right  aortic 
arch,  it  is  practically  a  continuation  of  the  distal  part  of  the  sixth  left 
arch  represented  by  the  ductus  arteriosus  to  which  its  origin  bears  a 
constant  relation. 

Right  Subclavian  from  Descending  Thoracic  Aorta. — In  this  anomaly 
the  aortic  arch  has  its  normal  course  to  the  left,  but  the  right  sub- 
clavian is  given  off  from  a  point  in  the  thoracic  aorta  just  below  the 
insertion  of  the  ductus  arteriosus  and  passes  up  to  its  normal  distribu- 
tion. Here  an  arrest  of  the  proximal  part  of  the  fourth  right  arch  which 
normally  forms  the  right  subclavian  has  occurred,  and  the  obliteration 


CONGENITAL  CARDIAC  DISEASE  437 

of  the  distal  portion  which  in  the  embryo  unites  the  fourth  arch  M^th 
the  aortic  trunk  has  not  taken  place. 

Common  Brachiocephalic  Trunk. — All  four  great  vessels  may  arise 
by  common  origin,  recalling  the  embryonic  stage  in  which  all  the  arches 
emerged  together  from  the  third  carotid  arch.  A  case  is  cited  by  Frey- 
berger.^ 

Clinical  Aspects. — The  evidences  presented  during  life  and  the  clinical 
significance  of  all  the  above  conditions  are  slight.  In  right  aortic  arch 
and  common  brachiocephalic  trunk  the  aorta  is  apt  to  rise  higher  toward 
the  neck  than  is  normal,  leading  to  violent  pulsation  in  the  episternal 
notch,  which  may  lead  to  a  mistaken  diagnosis  of  aneurism.  In  the  case 
of  origin  of  the  left  subclavian  from  the  pulmonary  artery  or  patent 
ductus  the  left  arm  usually  remains  entirely  free  from  cyanosis,  which 
shows  how  far  the  system  can  accommodate  itself  under  favorable  con- 
ditions to  an  admixture  of  venous  with  arterial  blood. 

ANOMALIES    OF    THE    CORONARY    ARTERIES. 

Anomalous  Origin  from  the  Pulmonary  Artery. — A  vessel  may  arise 
from  a  sinus  of  Valsalva  of  the  pulmonary  artery,  and,  meeting  the 
branches  from  the  aortic  coronaries,  produce  a  remarkable  anastomosis 
of  a  cirsoid  character.  In  Brook's  first  case,  a  vessel  the  size  of  a  crow- 
quill  sprang  from  the  right  anterior  sinus  of  Valsalva  of  the  pulmonary 
and  passed  down  over  the  infundibulum  of  the  right  ventricle,  there 
anastomosing  with  the  aortic  coronaries.  In  his  second  case  a  large 
anomalous  artery  arose  from  the  same  situation.  It  gave  no  branches 
to  the  heart  but  passed  to  the  left  and  upward  to  enter  a  complicated 
mass  of  thin-walled  arteries,  which  lay  around  the  main  pulmonary 
trunk  and  passed  up  along  the  trachea  and  behind  the  aortic  arch.  This 
mass  received  three  other  large  vessels,  one  from  the  left  subclavian, 
one  from  the  right  aortic  coronary,  and  one  from  the  posterior  aspect 
of  the  transverse  aortic  arch.    Krause's  case  is  similar. 

The  McGill  specimen  (see  Fig.  42),  was  from  a  woman  aged  sixty 
years,  v/ho  died  accidentally.  The  right  coronary  arose  in  its  normal 
situation  from  the  anterior  sinus  of  Valsalva  of  the  aorta  by  a  much 
dilated  orifice,  and  expanded  directly  after  its  origin  into  a  huge  thick- 
walled  loop  the  size  of  a  crab-apple,  which  projected  upward  some  2.5 
cm.  above  the  subepicardial  fat,  and  gave  off  the  descending  branches 
from  the  loop.  Both  these  and  the  main  trunk  of  the  vessel  were  wide, 
thick-walled,  tortuous  channels.  No  coronary  arose  behind  the  left 
posterior  aortic  cusp  in  the  normal  situation  of  the  left  coronary,  but 
instead  a  large  patulous  opening  lay  in  the  floor  of  the  dilated  posterior 
sinus  of  Valsalva  of  the  pulmonary  artery.  From  this  sprang  a  large 
thin-walled  trunk  of  venous  character,  which  divided  about  1  cm. 
beyond  its  origin  into  two  large  branches,  one  of  which  ran  to  the  left 
in  the  auriculoventricular  groove  in  the  course  normally  followed  by 
the  transverse  circumflex  branch  of  the  left  coronary  artery,  while  the 

1  Trans.  Path.  Soc,  London,  1898,  xliv,  44. 

2  Jour.  Anat.  and  Physiol.,  1902-03,  xxvii,  387. 


438 


DISEASES  OF   THE  CIRCULATORY  SYSTEM 


other  ran  downward  along  the  front  of  the  interventricular  septum  in 
the  position  of  its  descending  branch,  and  was  here  expanded  into  a 
large  triangularly  shaped  venous  sinus,  2  cm.  in  its  widest  diameter,  and 
diminishing  in  size  toward  the  apex.  In  the  floor  of  this  sinus  were 
several  thick-walled  septa  behind  which  large  vessels  opened  into  it  from 
the  myocardium. 

Fig.  42 


Aneurismal  dilatation  (arteriovenous  aneurism)  of  branches  of  coronary  arteries  in  a  case  of  anomalous 
origin  of  the  left  coronary  from  the  pulmonary  artery.  (From  a  specimen  in  the  Medical  Museum  of 
McGill  University,  Montreal.) 


The  cjuestion  of  the  circulation  in  the  anastomosing  vessels,  in  which 
blood  from  the  systemic  and  pulmonary  circulations  must  have  mingled 
is  of  interest.  Brooks  suggests  that  the  direction  of  the  current  must 
have  been  toward  the  cirsoid  aneurism  in  the  coronaries  arising  from  the 
aorta,  and  toward  the  right  ventricle  in  the  coronary  that  arose  from 
the  pulmonary  artery,  which  would  thus  drain  the  mass  and  would  also 
send  some  arterial  blood  to  the  lungs. 

In  the  McGill  specimen  the  peculiar  septa  in  the  floor  of  the  large 
venous  sinus  formed  by  the  descending  branch  of  the  anomalous  vessel, 
strongly  suggested  that  the  course  of  the  blood  was  toivard  the  pulmonary 
artery.  This  case  is  additionally  interesting  from  the  fact  that  the 
anomalous  vessel  was  here  clearlv  the  left  coronarv,  which  was  absent 


CONGENITAL  CARDIAC  DISEASE  439 

from  its  normal  situation  and  arose  from  the  pulmona^3^  Both  this 
and  Brooks'  second  case  were  in  elderly  subjects,  and  the  condition  had 
not  produced  any  manifestations  during  life. 

Miscellaneous  Anomalies. — Accessory  coronaries  may  be  present  or 
both  vessels  may  arise  behind  a  single  aortic  cusp,  or  there  may  be  a 
complete  absence  of  one.  A  case  has  been  recorded  of  an  anomalous 
coronary  sent  to  the  lungs  in  pulmonary  atresia. 


ANOMALIES    OF   THE   PULMONARY   ARTERIES. 

Accessory  Pulmonary  Artery. — ^A  series  of  10  cases  has  been  col- 
lected from  the  literature  by  McCotter,i  [^  which  an  anomalous  artery 
had  arisen  from  the  aorta  or  its  branches,  and  had  supnlied  the  lower 
lobe  or  the  accessory  lobe  of  one  or  other  lung.  In  the  case  reported  by 
himself,  in  a  man  aged  sixty-five  years,  this  artery  was  7  mm.  in  dia- 
meter, and  was  given  off  from  the  front  of  the  thoracic  aorta  on  a  level 
with  the  tenth  dorsal  vertebra,  and  passed  up  to  the  right  between  the 
folds  of  the  ligamentum  latum  pulmonis  to  the  lower  margin  of  the  right 
lung,  where  it  ramified.  The  lung  pleura  and  mediastinum  were  otherwise 
normal.  In  8  of  the  cases  collected  the  accessory  branch  was  from  the 
thoracic  aorta,  in  one  from  the  abdominal  aorta,  and  in  one  from  the 
seventh  intercostal  artery.  In  5  cases  the  accessory  branches  supplied 
an  accessory  lobe  and  in  5  the  lung  was  normal. 

Pathogenesis. — The  final  explanation  must  be  deferred  until  the  origin 
of  the  pulmonary  circulation  is  better  understood.  McCotter  gives  an 
interesting  discussion.  Accessory  pulmonary  arteries  have  been  described 
in  amphibia  and  reptiles,  and  are  said  to  be  normal  in  the  latter.  Thoma 
and  Evans  found  that  the  blood  \ascular  system  in  the  embryo  arises 
as  a  capillary  plexus  spreading  in  all  directions.  Such  a  capillary  plexus 
forms  caudally  from  the  pulmonary  arches  and  envelops  the  primitive 
lung  anlage  with  a  rich  capillary  plexus.  In  the  case  of  the  accessory 
pulmonary  branch,  this  plexus  must  have  formed  laterally  from  a  primi- 
tive thoracic  aorta  and  joined  the  pulmonary  plexus  just  as  a  capillary 
network  extends  to  the  limb-bud.  The  explanation  of  this  anomaly  is 
thus  either  (1)  that  this  plexus  always  occurs  but  has  failed  to  atrophy 
in  the  present  case;  or  (2)  that  the  plexus  is  only  occasionally  laid  down, 
i.  e.,  is  in  itself  an  anomaly,  and  when  present  results  in  an  accessory 
pulmonary  branch.  The  condition  has  not  shown  itself  to  be  of  any 
clinical  significance. 


ANOMALIES    OF    THE    SYSTEMIC   VEINS   ENTERING    THE 
HEART    AND    OF    THE    PULMONARY   VEINS. 

Systemic  Veins. — Persistent  left  suiMrior  vena  cava  is  the  commonest 
of  these  anomalies.  It  is  not  infrequent  in  conjunction  with  other  car- 
diac defects,  and  occurred  26  times  in  our  series.     It  is  of  little  clinical 

i^naL  i?ec.,  August,  1910,  291. 


440  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

importance  but  is  of  great  interest  in  cases  where  the  congenital  origin 
of  the  associated  condition  is  questioned,  as  indicating  the  developmental 
nature  of  the  latter.  The  cases  may  be  divided  into  two  groui3s :  those  in 
w^hich  the  right  superior  cava  is  also  present,  and  those  in  which  it  is 
absent,  and  the  blood  from  the  upper  portion  of  the  body  enters  the 
right  auricle  through  the  persistent  left  cava.  A  series  of  4  cases  of 
persistent  left  cava  is  published  by  Schutz.^  In  three  of  these  the  right 
cava  was  also  present,  in  one  it  was  equal  in  size  to  the  left;  in  a  second 
it  was  small  and  the  left  cava  communicated  with  the  left  auricle  by 
a  valvular  opening  in  its  wall  before  entering  the  right  auricle  at  the 
coronary  sinus.  In  this  case  the  apex  of  the  heart  was  bifid;  and  the 
patient  was  a  woman  of  thirty-eight  of  whom  no  other  history  was 
obtainable.  In  the  third  case  the  left  superior  cava  was  persistent  but 
rudimentary.  In  Schutz's  fourth  case  the  right  superior  cava  was  absent. 
The  left  innominate  veins  emptied  into  the  left  innominate  at  the  level 
of  the  left  common  carotid  to  form  a  left  superior  cava  thicker  than  a 
man's  thumb  which  widened  into  a  bulbar  swelling  4.5  cm.  across, 
which  entered  the  coronary  sulcus  and  opened  into  the  right  auricle 
above  the  inferior  vena  cava.  Habershon-  reported  a  case  of  absence 
of  the  superior  cava  in  a  man  aged  thirty-seven  years.  The  usual 
opening  of  the  superior  cava  in  the  right  auricle  was  marked  by  a  smooth, 
white  area  of  endocardium,  like  a  closed  foramen  ovale,  and  there  was 
extensive  development  of  collateral  circulation  through  the  vena  azygos 
major.  The  persistent  left  cava  was  formed  by  a  union  of  the  left  jugular 
and  subclavian  and  right  innominate  veins  and  emptied  into  the  dilated 
coronary  sinus.  The  recent  literature  on  complete  absence  of  the  right 
superior  cava  with  persistent  left  is  given  by  Dietrich.^ 

A  very  interesting  case  is  published  by  Beyerlein,^  in  a  boy,  aged  one 
and  a  quarter  years,  of  double  superior  vena  cava,  in  which  the  orifice 
of  the  coronary  sinus  in  the  right  auricle  was  obliterated  by  the  over- 
growth of  an  extensive  network  of  Chiari.  The  persistent  left  cava 
received  all  the  blood  from  the  coronary  veins  and  the  heart,  and  emerged 
from  the  coronary  sulcus  at  the  normal  situation,  emptying  into  the 
right  superior  cava  through  the  transverse  branch.  Two  cases  practi- 
cally identical  with  this  very  rare  anomaly  are  reported  by  Gruber^ 
and  LeCat  (quoted  by  Gruber).  Nabarro^  describes  a  case  of  double 
superior  cava  in  an  infant  of  three  months  where  the  persistent  left  duct, 
smaller  than  the  right,  was  joined  by  the  left  hepatic  vein,  which  emptied 
with  it  into  the  coronary  sinus.  Here  the  left  horn  of  the  embryonic 
sinus  venosus  had  evidently  escaped  obliteration. 

A  series  of  cases  in  which  a  displacement  to  the  left  of  the  superior 
cava  has  taken  place  so  that  its  orifice  comes  to  lie  directly  above  the 
interauricular  septum,   and  looks  into  both  auricles,  which  has  been 

1  Virchows  Arch.,  1914,  ccxvi,  35. 

2  Trans.  Path.  Soc,  Lond.,  1876,  xxvii,  79. 
^  Virchows  Arch.,  1913,  ccxii. 

4  Frank.  Zeit.  f.  Path.,  1914,  xv,  327. 

^  Virchows  Arch.,  1885,  xcix,  492. 

^  Jour.  Ajiat.  and  Path.,  1902,  xxxvii,  387. 


CONGENITAL  CARDIAC  DISEASE  441 

described  by  Ingalls  and  others,  and  a  similar  condition  of  the  inferior 
cava  by  Rokitansky,  are  described  under  auricular  septal  defects. 

Pulmonary  Veins. — An  anomalous  distribution  of  the  pulmonary  veins 
is  much  more  common  than  is  generally  supposed,  and  quite  serious 
deviations  from  the  normal  have  been  attended  with  surprisingly  little 
results.  Nevertheless,  their  displacement  occurs  in  many  complicated 
anomalies,  and  their  repeated  combination  with  these  grave  defects 
suggests  a  primary  error  in  development  in  the  pulmonary  veins  anlage. 
Quite  a  large  series  of  cases  of  biloculate  heart  are  reported  in  which 
the  pulmonary  veins  were  deflected  from  their  entrance  to  the  left 
"auricle  and  were  received  by  one  or  other  of  the  great  veins.  Schroeder^ 
gives  a  full  discussion  of  the  various  anomalies  of  the  pulmonary  and 
systemic  veins  and  traces  their  developmental  origin,  with  especial 
reference  to  those  cases,  like  his  own,  in  which  a  complete  defect  of  the 
interauricular  septum  was  associated.  Nabarro  describes  the  pulmonary 
veins  opening  into  the  coronary  sinus  in  an  infant  aged  five  and  a  half 
months,  in  whom  all  the  blood  from  the  systemic  circulation  must  have 
passed  through  the  patent  foramen  ovale. 

In  the  cases  reported  by  Ingalls,-  Chiari  and  others,  of  defects  at  the 
upper  part  of  the  interauricular  septum,  the  right  pulmonary  veins 
either  entered  the  right  auricle  or  the  superior  vena  cava.  In  those 
reported  by  Borst  and  Stoeber^  of  an  anomalous  septum  in  the  left 
auricle,  the  pulmonary  veins  entered  the  smaller  upper  chamber  in  the 
left  auricle  to  the  right  of  the  anomalous  septum,  which  evidently  rep- 
resented the  septum  primum,  deflected  to  the  left  by  the  entrance  of 
the  pulmonary  veins  too  far  to  the  right  side.  In  all,  the  primary  defect 
is  apparently  the  deflection  of  the  pulmonary  veins. 

Ramsbotham  describes  a  case  in  which  the  left  pulmonary  entered 
the  left  subclavian,  and  the  right  pulmonary  the  portal  vein,  and  in 
three  others  (Arnold,  Bochdalek,  Geipel)  the  right  and  left  pulmonaries 
entered  the  portal  vein  together  as  a  common  trunk.  The  pulmonary 
veins  of  both  sides  may  enter  the  left  auricle  as  a  single  or  as  two  trunks. 
Here  the  original  single  vein  has  not  been  taken  up  in  the  wall  of  the 
auricle  as  occurs  in  normal  development. 

The  clinical  significance  of  these  conditions  depends  less  upon  the 
defect  itself  than  upon  the  associated  developmental  conditions. 

DIAGNOSIS,  PROGNOSIS,  AND    TREATMENT  OF  CONGENITAL 
CARDIAC    DISEASES. 

Differential  Diagnosis. — In  the  diagnosis  of  congenital  cardiac  dis- 
ease two  questions  are  to  be  considered:  a  congenital  is  to  be  distin- 
guished from  an  acquired  lesion,  and  the  difi^erentiation  may  be  attempted 
of  the  particular  defect.  The  first  of  these  is  the  more  important  as  well 
as  the  simpler  problem.  It  is  necessary  both  for  a  wise  prognosis  and 
for  proper  treatment  to  recognize  the  congenital  nature  of  the  lesion, 

1  Arch.  filr.  klin.  Med.,  1911,  ccv,  122. 

2  Johns  Hopkins  Hosp.  Bull.,  1907,  xviii,  136. 
^  Virchoivs  Arch.,  1908,  cxciii,  252. 


442  DISEASES  OF   THE  CIRCULATORY  SYSTEM 

and  this  can  usually  readily  be  done.  The  following  conditions  are 
significant  of  the  presence  of  a  defect:  (a)  The  youth  of  the  patient. 
(b)  A  history  of  sj-mptoms  originating  in  early  childhood  or  in  infancy, 
and  of  the  absence  of  any  event,  as  rheumatism  or  endocarditis,  which 
could  have  led  to  an  acquired  lesion,  (c)  The  cyanosis  when  this  is 
present,  and  the  symptom  complex  associated  with  it.  (d)  The  presence 
of  atypical  physical  signs. 

The  diagnosis  of  the  various  defects  from  each  other  is  a  more  diffi- 
cult task.  In  some  of  the  most  complicated  forms  of  congenital  cardiac 
disease  both  signs  and  sj^mptoms  may  be  conspicuous  by  their  absence. 
And  on  the  other  hand,  several  anomalies  are  frequently  combined  in* 
the  same  case,  so  that  a  bizarre  picture  is  liable  to  be  produced,  even  in 
the  presence  of  marked  physical  signs.  Nevertheless,  a  careful  study 
of  the  literature,  and  the  application  of  this  at  the  bedside  has  convinced 
the  writer  that  in  the  great  majority  of  cases  auricular  and  ventricular 
septal  defects,  abnormal  communication  between  the  aorta  and  pulmonary 
artery,  patent  ductus  arteriosus,  subaortic  stenosis,  and  coarctation  of 
the  aorta,  all  of  which  conditions  are  characterized  usually  by  slight  or 
absent  cyanosis,  can  be  distinguished  from  each  other  and  from  pulmonary 
stenosis  or  atresia,  and  from  those  forms  of  biloculate  and  triloculate 
heart,  persistent  truncus  arteriosus  and  transposition  of  the  arterial 
trunks,  in  ivhich  the  cyanosis  is  a  more  conspicuous  feature.  This  state- 
ment has  the  authority  of  Hochsinger,  whose  special  work  along  the 
line  of  congenital  defects  convinced  him  that  the  differentiation  of 
cardiac  defects  is  largely  a  question  of  familiarity  with  the  clinical 
features. 

Cyanosis  is  the  rule  (to  which  a  few  exceptions  occur)  in  pulmonary 
stenosis  and  atresia,  in  complete  defects  of  the  septa,  as  biloculate  or 
triloculate  heart  or  persistent  truncus  arteriosus,  and  in  transposition 
of  the  arterial  trunks.  It  is  frequently  absent,  but  may  be  present 
and  this  especially  as  a  terminal  event,  in  patent  foramen  ovale  and  in 
defects  of  the  interauricular  and  interventricular  septa.  Cyanosis  is 
usually  absent,  except  during  dyspnoeic  attacks,  in  patent  ductus  and  in 
coarctation  and  hypoplasia  of  the  aorta. 

Dysimoea,  though  always  present  to  a  certain  degree  when  cyanosis 
is  advanced,  does  not  appear  to  bear  a  definite  relation  to  the  degree 
of  deficient  aeration  but  evidently  depends  on  some  other  factor  as  well. 
It  is  thus  characteristic  of  many  cases,  such  as  patent  ductus,  patent 
foramen  ovale,  or  septal  defects,  in  which  no  trace  of  cyanosis  is  seen, 
shortness  of  breath  and  palpitation  on  exertion  from  early  childhood, 
being  quite  frequently  complained  of.  The  same  is  true  of  dyspnoeic 
suffocative  attacks  with  transient  cyanosis  which  form  an  important  diag- 
nostic feature  of  such  cases. 

The  distinctive  character  of  \he  physiccd  signs  in  those  defects  which 
are  of  clinical  significance  have  been  discussed  under  the  individual 
lesions  but  may  be  briefly  summarized.  A  harsh,  systolic  inurmur  and 
thrill  localized  over  the  upper  part  of  the  precordium  and  of  diminished 
intensity  or  inaudible  at  the  apex  is  characteristic  of  pulmonary  stenosis 
and  of  septal  defects.    It  may  in  a  few  cases  be  heard  best  at  the  apex, 


CONGENITAL  CARDIAC  DISEASE  443 

and  it  may  vary  in  rhythm,  particularly  in  septal  defects.  Both  in 
pulmonary  stenosis  and  in  patency  of  the  duct  the  murmur  usually  has 
its  maximum  intensity  high  up  over  the  second  left  interspace  and  may 
be  heard  beneath  the  left  clavicle.  That  of  the  auricular  and  ventricular 
septal  defects  is  heard  over  the  third  and  fourth  left  interspaces. 
Murmurs  of  congenital  lesions,  when  heard  in  the  back,  are  usually  due 
to  patency  of  the  duct  or  to  septal  defects.  A  precordial  thrill  with  the 
same  localization  as  the  murmur  is  present  in  about  15  per  cent,  of 
congenital  defects  with  physical  signs,  its  presence  as  a  rule  corresponding 
to  the  degree  of  harshness  of  the  accompanying  murmurs. 

In  defects  of  the  inter  auricular  septum  the  murmur  is  often  post- 
diastolic or  presystolic,  and  in  patent  foramen  ovale  presystolic  and 
systolic  murmurs  may  combine  or  alternate  with  each  other,  and  may 
vary  with  change  of  position,  their  inconstancy  supplying  a  differential 
point. 

In  patent  ductus,  a  harsh  rumbling  machinery  murmur,  beginning 
toward  the  close  of  systole  and  continuous  throughout  the  cardiac  cycle 
is  present  in  a  certain  number  of  cases,  and  when  it  occurs  is  pathogno- 
monic; in  others  the  murmur  is  systolic  or  (rarely)  diastolic.  The 
pulmonary  second  sound  is  here  usually  accentuated  and  helps  to  differen- 
tiate patent  ductus  from  pulmonary  stenosis,  in  which  the  pulmonary 
second  is  usually  (not  always)  weak  or  absent.  An  abnormal  area  of 
dulness  above  and  distinct  from  the  cardiac  dulness,  in  the  first  and 
second  left  interspaces  (Gerhardt's  sign),  is  also  significant  of  the  latter 
stages  of  patent  ductus,  as  indicating  a  dilated  pulmonary  artery.  This 
sign  may  be  produced  also  by  retraction  of  an  atelectatic  left  lung 
(Hochhaus),  so  is  not  positive  unless  confirmed  by  the  a:-rays. 

A  powerful  diastolic  murmur  and  thrill  with  maximum  intensity 
over  the  second  and  third  interspaces,  and  an  accentuated  second  pul- 
monary sound  characterized  several  of  the  cases  of  abnormal  communi- 
cation between  the  lower  part  of  the  aorta  and  pulmonary  artery  (defect 
of  the  aortic  septum)  recorded. 

Complete  transposition  of  the  arterial  trunks  h^as  been  diagnosed  by 
the  absence  of  physical  signs  in  the  presence  of  marked  cyanosis,  and 
an  accentuated  pulmonary  second  sound  (Hochsinger). 

Coarctation  of  the  aorta  is  to  be  recognized  by  the  evidences  of  the 
collateral  circulation/ when  this  has  been  established,  hypertrophy  of 
the  left  heart,  the  frequent  association  of  an  acquired  aortic  insufficiency, 
and  a  reduction  in  the  force  of  pulsations  in  the  lower  extremities  as 
compared  with  the  upper. 

The  above  are  a  few  of  the  indications  by  which  typical  cases  may  be 
distinguished  from  each  other.  But  in  this  subject  it  should  be  remem- 
bered that  hard  and  fast  lines  may  not  be  drawn.  Thus  rhythm,  maxi- 
mum intensity,  transmission  of  murmur  or  thrill,  and  all  other  manifes- 
tations of  the  defect  will  be  found  to  vary  with  the  associated  cardiac 
conditions,  valvular  and  myocardial,  congenital  and  acquired,  far  more 
here,  where  so  wide  a  range  of  combinations  is  possibl^,^than  in  the 
acquired  forms  of  heart  disease.  For  this  reason  we  look  with  interest 
to  the  introduction  of  the  newer  methods  of  precision  in  this  difficult 


444  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

and  little  studied  field.  Several  positive  findings  have  already  been 
recognized  and  are  indicated  below.  There  is  good  reason  to  believe 
that  their  further  application  will  supply  us  with  a  more  exact  know- 
edge  upon  the  difi^erential  diagnosis  of  congenital  defects. 

Graphic  Methods. —  X-ray  Examination  and  Orthodiagraphic  Tracings. 
— Definite  information  upon  the  existence  of  hypertrophy  of  the 
ventricles  and  dilatation  of  the  auricles  is  to  be  obtained  from  the 
skiagraph.  Examination  of  the  shadow  at  the  base  of  the  heart  shows 
also  a  distinct  widening  in  the  presence  of  dilatation  of  one  or  other 
of  the  great  trunks.  When  the  pulmonary  artery  is  the  one  dilated  the 
widening  appears  as  a  distinct  bulging  on  the  left  side  just  above  the 
upper  border  of  the  heart,  forming  an  ".r-ray  cap"  in  the  position  of  the 
so-called  Gerhardt's  dulness.  Dilatation  of  the  aorta  is  indicated  by 
an  increased  shadow  to  the  right  of  the  median  line  in  the  same  situa- 
tion. Conversely,  hypoplasia  of  the  pulmonary  artery  is  indicated  by 
a  narrowing  of  the  shadow  to  the  left  of  the  heart's  base.  These  points 
and  the  typical  shape  which  the  ventricular  portion  of  the  heart  assumes 
in  the  various  valvular  lesions  are  well  shown  in  the  heart-silhouette 
obtained  by  the  orthodiagraphic  tracing.  Groedel^  has  figured  the  out- 
line obtained  both  in  the  various  acquired  valvular  lesions  and  also  in 
congenital  pulmonary  stenosis,  patent  ductus  arteriosus,  and  coarcta- 
tion of  the  aorta  and  points  out  that  in  patent  foramen  ovale  and  in 
ventricular  septal  defects  there  is  no  change  observed  at  the  site  of  the 
great  vessels  so  that  unless  hypertrophy  of  the  ventricles  has  occurred 
the  silhouette  is  normal. 

Fluoroscopic  Findings. — Deneke^  diagnosed  a  case  of  interventricular 
septal  defect  in  transposition  of  the  arterial  trunks  by  the  appearance 
on  fluoroscopic  examination.  The  heart  showed  a  moderate  degree 
of  hypertrophy  of  the  right  ventricle,  and  this  chamber  formed  the 
right  border  of  the  heart  in  place  of  the  right  auricle  as  normally  occurs, 
so  that  the  strong  pumping  movement  of  the  ventricle  could  be  seen  on 
the  right  border  as  well  as  on  the  left,  instead  of  the  fluttering  auricular 
movement  normally  seen  in  this  situation,  Deneke  describes  the 
appearances  as  follows:  "In  normal  hearts  the  movement  of  the  right 
border  is  seen  on  the  fluoroscope  as  a  sharp  auricular  twitching  preceding 
the  contraction  of  the  left.  Its  character  can  be  readily  distinguished 
as  auricular,  i.  e.,  a  short  fluttering  contraction  followed  by  long,  passive 
dilatation.  The  movement  on  the  left  border  is  slow,  lasting  much 
longer  than  that  of  the  auricular,  and  is  a  strong  pumping  motion  fol- 
lowed by  a  short  delay  in  the  contracted  state,  then  a  gradual  dilatation 
which  is  slower  than  the  ventricular  contraction,  but  much  quicker 
than  the  dilatation  of  the  auricles." 

The  Electrocardiographic  Curve  in  congenital  hearts  has  been  studied 
by  Lewis,^  Owen,^   Nicolai,^   Ratner,*^   Groedel   and  Monckeberg,^  and 

1  Deut.  Arch.f.  klin.  Med.,  1911,  ciii. 

2  Ibid.,  1906,  Ixxix,  38. 

3  Clinical  Electrocardiogra'phy ,  1913.  *  Heart,  iii,  113. 

5  Berlin,  klin.  Wchnsch.,  1911,  xlviii,  51.  ^  Berlin  Thesis,  1912. 

^  Cent.  f.  Herz.  u.  Gef.  Leid.,  January  1,  1913. 


CONGENITAL  CARDIAC  DISEASE  445 

others.  So  far  only  a  few  positive  points  have  been  elicited,  but  these 
are  sufficient  to  indicate  the  value  of  the  cardiogram  in  this  connection. 
(1)  A  "negative  initial  Schwankimg"  representing  a  deep  exaggeration 
of  the  S  wave,  in  lead  I,  has  been  observed  in  many  congenital  cases, 
and  was  described  by  Nicolai  and  Steriopulo  as  pathognomonic  of 
cardiac  defects.  It  is  merely  significant,  however,  of  the  extreme 
right-sided  hypertrophy  so  common  in  these  cases  in  which  both  right 
auricle  and  ventricle  are  involved,  and  is  seen  also  in  acquired  mitral 
stenosis  where  the  same  condition  of  marked  right-sided  hypertrophy 
and  dilatation  occurs;  as  a  corroborative  sign  it  is  often  of  use.  (2)  An 
extreme  amplitude  of  curves  in  several  leads  w^as  observed  by  Lewis  in 
congenital  cyanosis  and  is  described  by  him  as  a  "valuable  sign  of  con- 
genital valve  or  septal  defects."  (3)  Finally,  the  electrocardiogram  in 
true  (mirror-picture)  dextrocardia  when  the  heart  is  transposed  upon 
itself,  supplies  the  most  positive  sign  of  this  abnormality  that  we  possess. 
In  this  case  Lead  I  is  completely  reversed  upon  itself,  and  Lead  II  takes 
the  place  of  Lead  III,  exactly  the  same  tracing  being  obtained  as  when 
the  leads  themselves  are  reversed  in  a  normal  individual.  The  trans- 
posed electrocardiogram  decides  clearly  between  this  condition  and  a 
simple  dextroversio  cordis.  For  a  comparison  of  the  electrocardiograms 
in  these  two  conditions  (see  Fig.  22). 

The  polygraphic  tracing  may  show  a  positive  venous  pulse  of  mitral 
insufficiency  with  communication  between  the  two  sides  of  the  heart. 
In  the  absence  of  mitral  stenosis  (in  which  auricular  fibrillation  is  so 
common  and  may  give  rise  to  this  condition)  and  when  other  signs 
of  tricuspid  insufficiency  are  lacking  this  point  may  be  of  diagnostic 
value. 

Estimation  of  the  Oxygen  Content  of  the  Alveolar  Air. — An  important 
diagnostic  point  between  those  defects  due  to  abnormal  communications 
between  the  right  and  left  sides  of  the  heart,  and  those  due  to  pulmonary 
obstruction  has  been  supplied  by  Plesch,^  working  in  Ivraus'  laboratory. 
In  septal  defects  and  patent  ductus,  there  is  usually  an  admixture  of 
arterial  blood  with  the  venous  current  entering  the  lungs  through  the 
pulmonary  artery,  owing  to  the  fact  that  under  normal  conditions  the 
pressure  in  the  aorta  is  greater,  so  that  blood  passes  from  left  to  right 
through  the  defects.  Plesch  estimated  the  amount  of  oxygen  in  the 
alveolar  air  expired  from  the  lungs  which  he  obtained  by  his  method 
(described  by  Boothby  and  Peabody^),  and  found  that  in  these  condi- 
tions the  venous  blood  passing  to  the  lungs  is  reduced;  that  is  to  say, 
in  terms  of  percentage  of  its  oxygen  content,  the  latter  (O2)  is  raised. 
This  was  also  demonstrated  in  two  cases  of  Vaquez  disease  (polycy- 
themia with  splenomegaly)  in  which  the  oxygen  content  of  the  alveolar 
air  was  examined  by  Senator-Lowy  and  v.  Bergmann.  On  the  other 
hand  the  alveolar  air  in  cases  of  acquired  valvular  diseases  and  in 
one  of  congenital  pulmonary  stenosis  was  examined  and  showed  no 
deviation  from   the    normal.     These   figures   and   those   showing   the 

1  Berlin,  klin.  Wchnschr.,  1909,  xlvi,  392. 

2  Arch.  Iv,L  Med.,  1914,  xiii,  502. 


446 


DISEASES  OF  THE  CIRCULATORY  SYSTEM 


amount  of  oxygen  consumed  are  shown  in  the  table  taken  from  an  article 
by  Kraus.^ 


13 
E 

Polycythemia. 

CoDg 

enital  defects. 

Mitral 
stenosis. 

>i 

m 

V.  Bergmann's 
cases. 

•"0  S 
S  3  0 

-8 

ftT) 

m 

^2 

fl  0 

I. 

II. 

O2  used  pro 

3.52 

4.18 

4.02 

4.38 

4.83 

5.03 

6.74 

3.86 

4.62 

4.11 

kilo     and 

4.40 

minute. 

Reduction 

of  venous 

blood    in 

64 

83 

81 

64 

83 

86 

66 

64 

64 

62 

percent- 
age of  O2 

87 

content. 

The  differential  diagnosis  between  the  cyanosis  and  clubbing  of  con- 
genital cardiac  diseases  and  other  forms  is  discussed  under  cyanosis. 

Prognosis. — The  duration  of  life  has  been  considered  in  detail  in 
connection  with  those  defects  that  are  of  clinical  interest,  but  a  few 
generalizations  may  be  made.  The  prognosis  varies  with  the  lesion  and 
includes  a  wide  range  of  possibilities,  but  is  in  general  grave;  this  is 
based  upon  the  direct  interference  with  the  circulation  by  the  defect 
itself,  and  upon  the  well-known  tendency  of  certain  anomalies  to  become 
the  seat  of  a  future  malignant  endocarditis. 

Among  the  least  harmful  forms  of  congenital  cardiac  disease  may  be 
mentioned  anomalous  septa  in  the  auricles,  patent  ductus  arteriosus, 
and  coarctation  of  the  aorta  with  extensive  collateral  circulation,  which 
may  exist  until  past  middle  life  without  symptoms,  frequently  termina- 
ting then  with  a  general  failure  of  compensation  under  some  undue  strain. 
Localized  defects  of  the  interauricular  and  interventricular  septa  belong 
likewise  to  the  more  innocent  lesions  which  may  give  rise  to  symptoms, 
or  may  be  present  indefinitely  without  producing  any  effect  upon  the 
circulation,  becoming  serious  only  upon  the  advent  of  some  pulmonary 
complication  raising  the  pressure  in  the  right  heart,  or  through  the 
engrafting  of  a  malignant  endocarditis  along  the  edges  of  the  defects. 

In  the  more  complicated  defects  life  is  correspondingly  shorter. 
Young's  patient  with  cor  biatriarum  triloculare  and  anomalous  septum 
attained  the  age  of  thirty-nine  years,  and  Holmes'  twenty-four  years, 
but  these  are  rare  exceptions,  the  subjects  of  biloculate  and  triloculate 
heart  usually  dying  in  infancy.  This  is  true  also  of  persistent  truncus 
arteriosus,  although  a  patient  reaching  twelve  years  is  recorded  by 
Crisp.  In  pulmonary  stenosis  early  adult  life  is  not  uncommonly  attained, 
but  is  rarely  passed,  the  patients  dying  as  often  of  tuberculosis  as  of 
the  direct  effects  of  the  lesion.  Here  again  in  exceptional  cases  life  may 
be  prolonged,  Vulpian  recording  pulmonary  stenosis,  rechtslage  of  the 


Chart  from  Kraus'  article,  Berl.  klin,  Wchnschr,,  1910,  Ixxiv,  230. 


CONGENITAL  CARDIAC  DISEASE  447 

aorta,  and  defect  of  the  septum  in  a  man  who  died  at  the  age  of  fifty-two 
years.  The  average  duration  of  life  in  pulmonary  stenosis  is  fifteen 
years,  and  in  atresia  2.25  years  in  our  series. 

As  graver  conditions  proving  almost  inevitably  fatal  during  the  first 
weeks  or  months  of  life  may  be  enumerated:  complete  transposition  of 
the  arterial  trunks  without  defect  of  the  interventricular  septum,  pul- 
monary atresia  with  closed  interventricular  septum,  tricuspid  atresia, 
and  aortic  atresia,  which  is,  indeed,  the  most  serious  of  all,  nearly  all 
the  cases  recorded  dying  in  the  first  two  weeks  of  life,  and  many  within 
a  few  hours  of  birth.  The  same  is  true  of  most  forms  of  ectopia  cordis. 
Finally,  it  is  to  be  remembered  that  of  the  more  complicated  anomalies 
many  must  perish  in  the  early  stages  of  embryonic  development,  as 
only  those  in  whom  compensatory  conditions  arise  survive  until  birth. 

The  prognosis  depends  largely  upon  the  effects  of  the  lesion  upon  the 
circulation,  that  is,  upon  the  amount  of  deficient  aeration  produced, 
and  upon  the  compensatory  powers.  For  this  reason  symptoms  will 
frequently  prove  a  better  guide  to  the  immediate  future  than  physical 
signs.  Such  conditions  as  septal  defect,  for  instance,  may  give  marked 
murmurs  and  thrill,  yet  lead  to  no  hampering  of  the  heart's  action  and 
to  little  interference  with  oxygenation  until  some  additional  factor,  such 
as  obstruction  in  the  pulmonary  circulation  supervenes.  Persistent 
cyanosis,  a  continued  low  temperature,  a  marked  increase  in  the  number 
of  red  blood  cells  (above  5,500,000),  and  dilatation  of  the  heart,  all 
point  to  a  grave  disturbance  of  the  circulation  and  to  a  rapidly  fatal 
issue.  On  the  other  hand,  the  entire  absence  of  cyanosis  and  its 
attendant  phenomena  does  not  always  argue  a  favorable  prognosis, 
for  in  such  cases  sudden  death  may  occur  without  any  warning,  either 
quietly,  or  in  a  paroxysm  of  cyanosis  with  dyspnoea.  The  embarrass- 
ment to  the  circulation  which  the  lesion  itself  entails  is  not  the  only 
source  of  danger.  Grave  danger  lies  also  in  the  frequent  intercurrence 
of  a  malignant  endocarditis,  and  in  the  fact  that  infections  or  broncho- 
pneumonia are  apt  to  prove  rapidly  fatal.  The  liability  of  patients 
with  pulmonary  stenosis  to  tuberculosis,  and  the  frequent  termination 
by  sudden  cerebral  complications,  are  other  unfavorable  factors.  These 
considerations  indicate  the  extreme  gravity  of  the  more  pronounced 
cases,  and  the  fact  that  even  in  the  more  innocent  forms  of  congenital 
cardiac  disease  the  prognosis  must  be  framed  wdth  reserve  and  caution. 
Among  the  better  class,  where  good  hygiene  prevails  and  the  most  suit- 
able conditions  of  living  can  be  sought,  the  outlook  is  of  course  better 
than  among  the  children  of  the  very  poor. 

Treatment. — This  may  be  said  to  begin  with  the  care  of  the  mother 
during  her  pregnancy,  for  a  study  of  the  etiology  clearly  shows  that  to 
some  unhealthy  condition  in  the  environment  of  the  embryo  or  in  the 
parental  organism,  rather  than  to  an  ancestral  tendency  toward  anoma- 
lous growth,  the  majority  of  cardiac  anomalies  owe  their  origin. 

The  treatment  of  a  patient  suffering  from  congenital  cardiac  disease 
must  be  largely  symptomatic  or  palliative,  or  directed  to  the  preventing 
of  complications.  The  indications  here  are  to  do  all  that  is  possible  to 
facilitate  the  oxA^genation  of  the  blood,  to  avoid  additional  taxation  of 


448  DISEASES  OF  THE  CIRCULATORY  SYSTEM 

the  burdened  circulation,  and  to  shield  the  patient  from  accidents  or 
illnesses  which  increase  the  pulmonary  or  systemic  obstruction,  remem- 
bering always  that  in  the  majority  cyanosis  first  develops  on  the  addition 
of  some  such  factor  to  the  pathological  conditions  produced  by  the  lesion 
itself.  A  carefully  regulated  life,  a  plentiful  supply  of  light,  fresh  air, 
and  warmth,  the  maintenance  of  an  equable  bodily  temperature,  the 
avoidance  of  mental  agitation  and  of  undue  physical  exertion,  rest,  and 
quiet  forms  of  exercise,  where  this  last  is  permitted  by  the  condition  of 
the  patient,  are  all  essential.  The  diet  should  be  carefully  ordered, 
light  and  nutritious,  and  the  often  capricious  appetite  watched.  Free 
action  of  the  excretory  organs,  especially  of  the  skin,  should  be  promoted 
and  the  child  kept  clothed  with  flannel.  Sudden  changes  in  the  external 
temperature  must  be  avoided  and,  when  possible,  resort  should  be  had 
to  a  warmer  winter  climate.  Exposure  to  cold  or  wet,  or  to  any  of  the 
causes  of  rheumatism,  should  be  avoided  on  account  of  the  great  liability 
to  acute  endocarditis.  When  adult  life  is  attained,  choice  of  light 
employment  which  does  not  call  for  sudden  or  great  physical  exertion  is 
important.    In  women  child-bearing  is  fraught  with  danger. 

Operative  interference  in  patent  ductus  arteriosus  in  the  form  of 
ligation  of  the  duct,  was  suggested  by  Munro^  on  the  ground  that  a 
probable  diagnosis  is  now  possible  and  that  the  vessel  lies  in  an  accessible 
situation.  The  fact  that  distinctive  signs  occur  only  after  pulmonarj^^ 
dilatation  has  taken  place  and  a  certain  adjustment  of  the  vessels  to  the 
new  order  of  the  circulation  has  set  in,  would  make  one  hesitate  to 
resort  to  so  radical  a  measure,  which  might  introduce  a  new  factor  of 
disturbance. 

When  cyanosis  has  developed,  the  administration  of  oxygen  has 
been  suggested  as  likely  to  be  useful  in  relieving  dyspnoea.  Gibson  and 
others  report  a  negative  result  from  its  use  in  several  cases.  For  the 
relief  of  the  dyspnoeic  attacks  diffusible  stimulants  are  of  benefit  and 
'should  be  kept  at  hand;  and  in  infants  the  hot  mustard  bath  is  useful. 
The  frequent  syncope  may  best  be  relieved  by  strychnine.  When  failing 
compensation  sets  in,  the  usual  treatment  of  rest  and  cardiac  tonics  is 
to  be  employed,  and  here  strychnine  is  said  to  give  better  results  than 
digitalis. 

Thus,  in  a  very  few  words,  a  careful  hygiene  and  an  expectant  and 
preventive  treatment  may  be  summed  up  as  the  only  available  assistance 
that  can  be  given.  The  condition  does  not  admit  of  cure,  but  permits 
of  amelioration  and  of  arrest  of  the  downward  trend  of  the  disease. 

1  Ann.  Surg.,  1907,  xlvi,  335. 


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